Johnny "Joe Kid" Joestar was born in a family of former aristocrats in Danville, Kentucky – his father then a wealthy owner of several farms and an acclaimed horse trainer who had won the Triple Crown seven times. He was successful enough for the family to live comfortably in England for several years.
Johnny was five when he first rode a horse. His father, upon seeing Johnny observe the motions of the horse, considered him "a horse riding genius", which Johnny himself would also believe. However, Mr. Joestar was strict towards Johnny and always seemed to favor his older brother Nicholas.
When young Johnny took out his pet mouse Danny and secretly fed it under the table during dinner, his father, upon discovering it, ordered Johnny to drown the animal as punishment. Heartbroken, Johnny was unable to bring himself to do the deed, and followed Nicholas' suggestion to set Danny free into the woods instead.
Years later, Nicholas grew into a promising jockey-to-be, much admired by Johnny. One day, Nicholas' horse stumbled from something on the ground, costing Nicholas his life. Johnny saw a white mouse run from the commotion into the woods – a mouse he believed to be Danny.
Seven years later, it was Johnny who rose to fame as a racing prodigy. He was pampered greatly: people would bring him riches, politicians and royalty would visit him, and rich girls would sleep with him. His father still did not acknowledge him and his feats, possibly because Johnny never managed to beat Diego Brando at racing. In a violent fight between them, Johnny's father uttered that "God had taken the wrong son" before finally throwing Johnny out of the household. Still, this did not deter Johnny, and he would go on to win the Kentucky Derby at a tender age of sixteen.
At one point, while waiting in line for a play, the girl he was with convinced him to cut to the front of the line and bribed the bouncers to throw out the youth in the front of the line - who had been waiting through the night. The youth shot Johnny in the spine, paralyzing him from the waist down and sending him to a hospital where he was physically abused. Having lost all the friends and respect he had earned as a jockey, no one visited him during his stay in the hospital.
Johnny first appears among the crowd who witness the confrontation between Gyro Zeppeli and a pickpocket who had stolen money from him. Afterward, when Johnny follows and touches the Steel Ball Gyro had used, which was still spinning, his legs moved enough to allow him to stand. Johnny chases after Gyro, who compliments his upper body strength, but denies that the Steel Ball can enable him to walk again. However, Johnny is not deterred; he decides to chase down Gyro by entering the race to learn more about the Steel Balls and starts by trying to ride a horse.
Johnny's initial attempts are unsuccessful, the experienced horse dragging him by the saddle in the dirt, causing his leg to be pierced by a piece of wood, and even trampling him through an entire day's attempt at trying to tame it. Johnny also made sure not to be interrupted in his attempts to tame him, threatening the men who introduced him to the horse that he would set himself on fire and commit suicide if he is forced out of the pen.
Johnny enters the race, Slow Dancer dragging him along the stirrup. However, it becomes evident that Johnny has tamed the horse, as it is seen licking his cheek and obeying Johnny's command to lick him again. Johnny, after taking Gyro's hint to use the spin, rolls back over Slow Dancer's neck and onto the saddle, right as the Steel Ball Run starts.
During the first stage, Johnny steadily follows Gyro, determined to impress him during this stage. When Gyro decides to cross a dense forest to take shortcut, he decides to dash in too, clashing with Pocoloco briefly. While Johnny has superior skill, Pocoloco's luck sends him to the second position nonetheless.
Until the final 1,000 meters, Johnny remains unchallenged until Diego comes behind him. Nonetheless, Johnny finishes at the fifth place. Still determined to follow the Neapolitan rider, Johnny impresses Gyro enough for him to give him a second lesson in the Spin. Telling him that the secret resides in the rotation, Gyro gives Johnny a rock to rotate and forms a partnership with him.
At the start of the second stage, Johnny counsels Gyro to follow the advised route linking all the possible wells during the crossing of the desert. However when Gyro decides to cut through 150km of desert, Johnny has no choice but to follow him.
Johnny and Gyro are attacked by one fellow competitor, Mrs. Robinson, who manages to blind and unhorse Johnny, but Gyro manages to save him and defeat Mrs. Robinson.
At night, they are again attacked, this time by the Boom Boom Family, whose magnetic power disable Gyro's Steel Balls and Johnny's pistol. The duo opt to flee, but Benjamin Boom Boom the patriarch lures bounty hunter Mountain Tim toward thinking Johnny has been assassinating his competitors. When Mountain Tim, Johnny and Gyro meet, the magnetic power of Tomb of the Boom immobilizes the three of them. When Benjamin moves to kill Johnny, the later acquire the ability to rotate his fingernails. He shoots the nails, killing Benjamin and crippling L.A. with his newfound power, which Mountain Tim explains as a Stand. Since the true assassins were dealt with, Johnny and Gyro resume their race, but L.A. grimly warns Gyro that he will never finish the race as other people are after his head.
Johnny pushes Gyro to reveal his past as a Neapolitan executioner, whose effort to win the Steel Ball Run are undermined by enemies of his kingdom. One such enemies reveals himself as another Stand user. Oyecomova, a Neapolitan terrorist, battles Gyro and Johnny but both cooperate in order to take him down.
Even as he has to deal with the threat of terroritsts, Johnny discovers that a mummified Left Arm has entered his own body. Another enemy different from the terrorists makes his entry. The Pork Pie Hat Kid, a Stand user at the service of the President of the United States disables Gyro and tries to kill Johnny. During the fight, Johnny witnesses his Stand making his legs move, and realizes how precious those Corpse Parts can be to him. Johnny and Gyro's concerted efforts manage to prevail against the Pork Pie Hat Kid; Gyro though interested in the Corpse Parts, has nothing to do with them.
Johnny and Gyro peacefully finish the second stage, Johnny ending up in second place as he spots in the newspaper a clue to the next Corpse Part.
In the Rocky Mountains, Johnny and Gyro meet with an unhorsed and visibly hurt Diego. Johnny advocates leaving Diego, but the British jockey soon regains his vitality and acts strange. The three of them decide to spend the night in a village to avoid the cougars, but Johnny and Gyro soon have to deal with Diego, who transformed into a feral dinosaur.
Both escape, but have been scratched by the dinosaur and slowly begin their own transformation. Noticing that Johnny's metamorphosis into a dinosaur is slowed by the Corpse Part, the duo try to reach the new Corpse Part on a nearby peak. However both are stopped at the last moment by Diego and the horde of dinosaurs, which was commanded by Dr. Ferdinand, another US agent. Johnny, immobilized, relinquishes his Left Arm in order to transform and use his newfound mobility to give Gyro one of the Eyes. Gyro, now immune to Dr. Ferdinand's power, manages to defeat the doctor while Diego flees with the other eye.
Johnny and Gyro then ride safely toward the end of the third stage. As every competitor races to finish first, Gyro uses yet another dangerous shortcut, but Johnny admonishes him, criticizing his lack of real, selfish drive he needs to come out on top. At first, Gyro is angry at Johnny and criticizes him, but after being defeated in the race by Diego, decides to listen to Johnny's words.
The fourth stage begins for Johnny and Gyro. They ride peacefully for about 600 km.
In a forest, Johnny and Gyro meet Hot Pants, a competitor who won the previous stage. Hot Pants attacks them both with his Stand Cream Starter, believing they stole one of his stash of food. Fortunately, Johnny defuses the fight by demonstrating they couldn't have stolen it. Johnny, Gyro and Hot Pants are still on bad terms but have to cooperate as a gunslinger named Ringo Roadagain blocks their way out of the woods.
Hot Pants and Johnny are wounded, Johnny receiving a bullet to the head, but the near unconscious Johnny manages to signal to Gyro that he's still alive. Lying near Ringo, he witnesses the gunslinger scribing a message and give it to a messenger pigeon. Gyro comes challenging Ringo to a gunfight, and prevails. Then healed by Cream Starter, Johnny warns Gyro about Ringo's message, which contained the coordinates of the next Corpse Part. Also checking on Hot Pants, Johnny sees that Hot Pants is secretly a woman. Deeming her gender irrelevant, Johnny chooses not to disclose this to his partner.
Near Kansas City, Johnny and Gyro meet Sandman who warns them of an incoming tempest. Yet, when Sandman informs them of Diego's route, they decide to run after him. After a tough battle, Johnny and Gyro manage to outpace him, and Diego's Silver Bullet is injured, putting him temporarily out of the race.  However they are surprised to meet Lucy Steel, who had discovered Valentine's schemes and is now pursued by Blackmore. After Blackmore is killed, Lucy gives them the Spine and asks for their protection. Instead, Gyro wants Lucy to go steal the Heart from Valentine, exploiting that Lucy's involvement was known only to Blackmore. Despite Johnny's protests at sending her, she accepts.
Johnny and Gyro finish the fourth stage in Kansas City, then see Diego crossing the line, sure that he will get back at them eventually.
En route for Chicago, the next finish line, Johnny and Gyro ride peacefully for around 300 km.
Near the Mississippi, they are attacked by two unknown opponents. Johnny's nails are too weak while Gyro's Steel Balls are destroyed, thus they opt to flee to the river. They soon discover that Diego and Sandman are cooperating, and while Diego has gone, Sandman has cornered them in the middle of the river. As they fight, Johnny reminisces how life was harsh to him, and is driven to despair. However, Gyro refuses to give in and teaches Johnny about the Golden Scale. Showing Johnny his belt buckle as a scale, Gyro nonetheless refuses to give it to him. In the middle of the Mississippi, as Gyro's leg is chopped off, and Johnny is on the brink of despair, the latter finally understand that the Golden Scale is omnipresent in the surrounding nature. Tusk evolves into a new ACT, which enables him to create enduring holes. Although it isn't enough to kill Sandman, Johnny uses Gyro's belt buckle as an extra projectile to kill him. Johnny is victorious, but in his weakened state, he cannot stop Hot Pants from stealing the Corpse Parts; still Hot Pants left him a Vertebrae and helped the duo cross the Mississippi.
At the beginning of the sixth stage, Johnny and Gyro meet with Sugar Mountain, a young girl acting as the guardian of a giant tree in a forest. Soon the duo has to face strange propositions between two versions of various objects they dumped in the nearby ponds, the real items and a much more precious version of them. They answer honestly and get both, until Gyro and Johnny manage to obtain the Ears and the Right Arm. However Sugar Mountain also tells them to trade everything they have by sunset fairly otherwise they will be absorbed into the tree.
In a nearby city, Johnny and Gyro have trouble spending everything they have, moreover Eleven Men stalk them in order to assassinate them both. Gyro decides to go to an underground casino in which they try to gamble away the money. They are interrupted by the Eleven Men, and a shootout ensues. Overwhelmed by numbers, Gyro manage to convince the gangster to kill the Eleven Men in exchange for all their goods. All but one die, the last having fled.
As the sun sets, Gyro suddenly transforms into a tree, to Johnny's surprise. He realizes with horror that he must trade the Corpse Parts, but as Gyro transforms and begs Johnny to trade them away. Johnny clings to the Corpse Parts who means the potential use of his legs. Still, at the last moment, Johnny trades the Ears and the Right Arm with the last of the Eleven Men, crying as he asks for a half empty bottle of wine in exchange for the Corpse Parts. Having saved Gyro, Johnny share the bottle with him and has a toast to the next Corpse Part and the goal.
When Johnny and Gyro attempt to cross the Michigan Lake via a frozen strait, they notice a wolf following them, but also two other agents of the government: Wekapipo and Magent Magent. Wekapipo's expertise in the Spin proves problematic, but Johnny and Gyro manage to prevail. When Gyro reveals that his family is taking care of Wekapipo's blind sister, the latter allies with them and accepts to go look for Lucy. Moreover, Johnny obtains the Legs from the wolf.
Being in a victorious stroke, Johnny and Gyro finish the stage at the second and first place respectively.
Johnny and Gyro ride undisturbed for 12 days, until they are 145 km for Philadelphia.
Johnny and Gyro spot Hot Pants, and follow her in order to get the Corpse Parts she stole back. When she enters a garbage dump, Johnny and Gyro separate themselves to surround her, but Hot Pants manage to warn Johnny about the new enemy's power, who lured Johnny and Gyro into his Stand. Civil War summoning copies of whatever and whoever the victims left behind, but weak to water, Johnny manages to escape it and goes back into the dump to save Gyro. Seeing visions of Jesus, Johnny's Stand evolves further into ACT 3, and though Axl RO takes all the Corpse Parts, Johnny manages to return his power against him.
Defeated, Johnny and Gyro finish the sixth stage at the sixth and seventh place.
In Philadelphia, Johnny and Gyro have no Corpse Parts anymore and decide to steal the Right Eye from Diego, following him up to a park.
However, Valentine manages to shoot Johnny, forcing him to escape into the sewers while his Stand, Dirty Deeds Done Dirt Cheap, causes confusion among Gyro, Diego and Wekapipo. Valentine manages to escape with Diego's Eye, but Johnny now knows Valentine's power.
Despite Gyro's reluctance at continuing a seemingly lost fight, Johnny briefly stands up, showing that he's this close to regaining the use of his legs. Thus Gyro accepts to follow Johnny, mentioning another level of Spin which could defeat Valentine.
Johnny and Gyro follow Diego and Hot Pants who have allied themselves, and observe the two racers battle Valentine on board a train from afar. When Diego dies, they decide to intervene and stop the train from the locomotive. However, they also notice that the landscape is converging toward the train. A brief shootout ensues between Valentine and Johnny, but Valentine discovers the blessing of the Corpse, a pocket dimension on which "harm" itself bounces away. Valentine almost kills Johnny but Gyro throws a Steel Ball at the wall, making the President retreat momentarily while Johnny and Gyro manage to stop the train and take a respite. Gyro's Ball has managed to momentarily cross the wall; the Super Spin, a level of Spin reached only when a horserider manages to ride within the Golden Scale, seems to be able to cross over dimensions. The duo now charges at the President with the intent of using the Super Spin, and Valentine dashes between them, forcing Johnny to separates from Gyro. Although he momentarily manages to reach the Golden Scale, Johnny hits a tree branch, giving Valentine the occasion of cutting off his hand. However, a combination of Gyro throwing one of his Steel Balls and Steven Steel entrusting Lucy with the Neapolitan distracts Valentine away from Johnny.
While Gyro and Valentine duel, Johnny recomposes himself. As he tries to join the fight, Gyro shouts to Johnny his last lesson: "The shortest route was a detour; it was the detour that was our shortest path". Gyro then dies by Valentine's hands. Heartbroken and enraged, Johnny charges Valentine, but the President manages to slit Slow Dancer's throat and unhorse Johnny. As Valentine approaches him to deal the last blow, Johnny understands Gyro's message, and uses the Steel Ball on Slow Dancer's hind leg. Kicked into the air, Johnny absorbs the Golden Scale into himself and unlocks Tusk ACT 4, and shoots his last nail toward Valentine. Valentine is hit, and disappears momentarily.
Johnny waits for the President's eventual return, growing back the nails of his only hand. When Valentine does come back, he tries to sway Johnny into releasing him from the infinite rotation caused by Tusk, promising to bring back Gyro and leave them be, also making an elaborate speech about his goals and giving an alternate Cream Starter as insurance. Almost convinced, Johnny nonetheless tosses Valentine's old revolver his double discarded earlier, exposing Valentine as treacherous. A small duel ensues, during which Johnny kills Valentine, and sees Gyro's spirit giving him a last goodbye.
Ninth Stage and Final Duel against Dio
After Johnny finally kills Valentine, he is surprised to see that the Corpse Parts have gone missing again. He pursues the thief, only to discover it is none other than Diego Brando (or rather, a Diego from an alternate world). Johnny is at first surprised by the appearance of Diego's new stand THE WORLD, but still manages to discover the nature of its power after a few attacks. This time, however, Diego has the advantage of already knowing Tusk's powers and the super spin technique, predicting Johnny's actions and using his own fangirls as human shields. In his final attack, Diego uses his "5 seconds" to create a pincer move: he fires a gun from the front while THE WORLD attacks Johnny's back. Johnny is badly hurt again and falls from his horse, unable to use his new attack.
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However, he had accumulated enough spin to make one final attack, sending Tusk Act 4 climbing up Diego's leg. Diego then remembers what Valentine had told him about the Super Spin technique and how to overcome it. Cutting his leg off and throwing it at Johnny, Diego manages to beat the Spin User with his own technique. He then remounts his horse and leaves Johnny to die. Having lost the race, the corpse and now dying as a result of his own attack, Johnny nearly sinks into despair. However, before his life can come to an end, Steven Steel appears. With Steven's help (riding his horse to do the spin), Johnny performs Tusk Act 4's infinite spin power on himself to reverse its effects. Johnny is never seen completing the ninth and final stage of the race and ends with a total of 295 points.
Post Steel Ball Run
After the race, Johnny travels by ship to Naples in order to return Gyro's corpse to his family, intending to honor him one last time. On the ship, he meets Norisuke Higashikata and his daughter Rina and begins a romantic relationship with her. It is revealed in the aftermath that the monarchy is overthrown and Marco is granted amnesty, though he dies of a cold later.
Last years in Japan
According to family tree left in the 1987 edition of Norisuke Higashikata I's record of the Steel Ball Run race, Johnny had married Norisuke's daughter Rina Higashikata in 1892 and had a son and daughter with her. Johnny and Rina first lived in a farm, then Johnny came to Japan by request of the Japanese government to teach horsemanship, and also contributed to the importation of foreign fruits. According to the old man on Shakedown Road, about nine years after the marriage, Rina was stricken with an incurable disease that gave her on-and-off amnesia and origami-like folded skin. Johnny thus brought Rina back to Morioh so that she lived in her native town.After agonizing over his wife's condition Johnny turned to stealing the Corpse from the vault and brought it back to Morioh to cure her. The American government reacted from having the Corpse taken and pursued Johnny, but by taking advantage of a certain privilege and sneaking aboard a ship crossing the Atlantic and Indian oceans, he narrowly evaded them into the Pacific.With the government pursuers still following, Johnny took the Corpse and hid it at the roots of the Meditation Pine by the coast of Morioh and then fetched Rina and their infant son, George, by carriage. Using the Holy Corpse's power to cure disease (more accurately described as a power to remove), the curse was removed from Rina, but was unfortunately forced upon George instead. Having promised not to use the Corpse anymore, but willing to use it again to cure his son, Johnny mounted his horse and placed his child atop the case containing the Corpse. After gaining a certain amount of velocity, Johnny activated the Super Spin and shot his son and the Corpse at the same time using Tusk Act 4. He took the curse upon himself and was shot in the head by his own Stand. After falling to the ground, the Stand Les Feuilles manifests itself for the first time and transfers a large boulder on Johnny's head, crushing his skull. Thus Johnny Joestar's life ends at the age of 29, in the evening of November 12th 1901. However, these details remain a rumor.
His body is discovered the next morning by Rina next to George and the case containing the Corpse. Due to the lack of witnesses, everyone believed Johnny had been murdered and the prime suspect was his wife, Rina Higashikata, who was released later when the incident was classified as an accident. Six months later, a Jizou statue was erected in memory of Johnny Joestar and he was buried in Morioh.