To report a case of endovascular aneurysm repair (EVAR) in a patient with severe hemophilia B.A 63-year-old man with severe hemophilia B was referred for the treatment of a symptomatic infrarenal aortic aneurysm that had enlarged from 3.1 to 4.2 cm in diameter in the last 6 months. The patient was successfully treated with EVAR, associated with perioperative administration of titrated doses of high-purity factor IX concentrate. There were no perioperative complications, but a small type II endoleak was found on the initial postoperative computed tomographic scan; on the 6 month scan, the endoleak had not changed, and it is under intensive surveillance.This case demonstrates that endovascular abdominal aortic repair is safe and feasible in severe hemophilic patients managed perioperatively with deficient coagulant protein replacement.