Background <p>Takayasu arteritis (TAK) is a granulomatous large-vessel vasculitis typically affecting young adult females. Pediatric cases are rare, and infantile onset is exceptional. Management relies on immunosuppression, with surgery reserved for severe complications.</p> Case report <p>We describe a now 5.5-year-old boy diagnosed with TAK at six months of age, presenting with hypertensive encephalopathy and kidney dysfunction. Despite treatment with corticosteroids and anti-TNFα, his kidney function deteriorated, leading to kidney failure and dialysis. At nearly three years of age, he underwent abdominal aorto-aortic bypass and bilateral nephrectomy due to progressive vascular narrowing and refractory hypertension. At age four, he successfully received a deceased-donor kidney transplant. Eighteen months post-transplant, he maintains excellent graft function and shows no signs of TAK recurrence.</p> Clinical significance <p>This case underscores the complexity of diagnosing and managing infantile TAK with multiorgan involvement. To our knowledge, he is among the youngest reported TAK patients to undergo successful kidney transplantation following major vascular surgery. His course demonstrates the potential for long-term remission and safe transplantation under standard immunosuppression, without continued anti-TNFα therapy. The literature is sparse regarding kidney failure and transplantation in TAK, particularly in infants.</p> Key management points <p>This case highlights key management dilemmas in infantile TAK, including clinical diagnosis, timing of surgery and transplantation, choice of immunosuppression, and long-term monitoring. It emphasizes the importance of a multidisciplinary approach and the need for collaborative research to address knowledge gaps in this rare but complex condition.</p> Graphical abstract <p></p>