Horseshoe kidneys and their wide-ranging anatomy present a unique test to the open surgical approach for repair of abdominal aortic aneurysms. Endovascular options are currently the desired strategy for treatment of abdominal aortic aneurysms and offer multiple advantages when horseshoe kidneys are present. Even so, the challenging nature of these patients demands a high degree of caution and planning. This case details a patient with horseshoe kidney who underwent percutaneous endovascular aneurysm repair for an abdominal aortic aneurysm and developed isthmus infarction early in the postoperative period with an uneventful subsequent recovery. Here we report what we believe is the first successfully described percutaneous endovascular aneurysm repair to treat a patient with horseshoe kidney.