Surgical repair of an abdominal aortic aneurysm (AAA) concomitant with a horseshoe kidney (HSK) may be technically demanding because of the complex anomalies of the kidney and of its collecting system and arteries, the greater risk of HSK-related complications, and the often unexpected intraoperative finding of HSK itself. We reviewed a database of more than 500 patients with AAA observed in our surgical department from 1994 to the time of writing. Five patients had AAA concomitant with HSK. Two of these patients did not undergo surgery because of the small dimension of the aneurysm or because of their poor health. The other three underwent successful repair of AAA with different techniques; namely, an aortobifemoral bypass via a thoracoabdominal retroperitoneal incision in one, a straight graft via an emergency median laparotomy in one, and an endovascular repair followed by open surgery 4 years later for endotension in one. Abnormal minor renal arteries were deliberately occluded and only one of these caused a minor renal infarct, but without functional impairment. These data and a review of the literature indicate that HSK should not preclude repair of coexistent AAA, as imaging procedures provide the information necessary to plan the best approach for each patient. Up-to-date surgical procedures, a posteriori retroperitoneal approach or endovascular repair, and deliberate occlusion of the minor renal arteries appear feasible and safe as they avoid most of the anatomical problems and provide results equivalent to those of uncomplicated aortic surgery.