Patient: Male, 54-year-old Final Diagnosis: Giant right renal artery aneurysm Symptoms: Bilateral leg edema Medication: — Clinical Procedure: — Specialty: Hematology • Radiology • Urology Objective: Rare coexistence of disease or pathology Background: Classical hemophilia, or hemophilia A, is an X-linked recessive genetic disorder characterized by deficiency in clotting factor VIII. Renal artery aneurysms (RAAs) are also rare and are defined as a focal dilatation of the renal artery that exceeds 1.5 cm in diameter. These 2 rare conditions – giant RAA and hemophilia A – were simultaneously observed in our patient. This report presents a male patient with hemophilia A with a 10-cm aneurysm of the right renal artery, which was treated with transarterial coil embolization and factor VIII infusion. The giant RAA was an incidental finding and was suspected after the abdominal ultrasound (US). Case Report: We present the case of a 10-cm right RAA in a 54-year-old man with hemophilia A. The patient had a congenital severe coagulation factor VIII deficiency (hemophilia A). He presented at a routine hematologist visit with an atypical symptom of severe symmetrical leg edema. Laboratory tests showed increased levels of creatinine and proteinuria. Investigations proceeded with computed tomography (CT) and digital subtraction angiography (DSA). Endovascular coiling of the aneurysm was performed with perioperative recombinant coagulation factor VIII substitution, and the recovery was uneventful. At 6-year follow-up there are no signs of proteinuria, and kidney function was stable. Conclusions: We present a case of renal artery aneurysm effectively treated by endovascular embolization, showing the importance of managing patients with hemophilia A according to a guidelines-based multidisciplinary approach and ensuring the lowest possible risk of peri- and intraoperative complications by using minimally-invasive treatments.