Abstract: Surgical repair of abdominal aortic aneurysms (AAAs) with coexisting horseshoe kidney (HSK) is technically challenging because of complex anatomy. Endovascular repair has emerged as a common approach to AAA repair and represents a valid alternative in selected patients with AAA and concomitant congenital renal anomalies. We report a case of successful percutaneous endovascular repair in a patient with coexistent AAA and HSK. Careful preoperative planning is essential in successful endovascular management of abdominal aneurysms in these patients.