Background: Takayasu arteritis (TA) is a large vessel systemic vasculitis, affecting the aorta and its main branches, leading to stenosis, thrombosis, and aneurysm formation. Incidence of 2.6 cases per million per year has been reported. TA is rare condition in children. Steroids are the main therapy for active disease, however, additional immunosuppressive agents are required in 50% of the patients. Objective: To report a case of TA in a young infant, presenting with intestinal necrosis. Case Report: 12 month-old male diagnosed with TA at 6 month-old, treated with second line immunosupressors. He presented with 3 days of abdominal pain, images showed aneurysm and thrombosis of the superior mesenteric artery. Small bowel ischemia was confirmed. The patient underwent multiple surgical interventions with bowel resection, leaving 30 cm of jejunum and 10 cm of terminal ileum. Anticoagulant therapy was started immediately. Intestinal continuity was restored 6 weeks later, patients was discharge home 3 months after surgery in full enteral intake, anticoagulant therapy, and maintains immunosupressor therapy. Discussion: TA in young infants is uncommon, and they can present atypical clinical manifestations. These patients are a real challenge, requiring multidisciplinary care to avoid further morbidities related to the vasculitis.