Abdominal aortic aneurysm (AAA) is rarely associated witha congenital pelvic kidney. To date only 11 cases have been reported in the literature in which a solitary pelvic' kidney was associated in only 1 patient. Repair of thesaneurysm is technically demanding because the abnormal origin of the renal arteries presents the problem of renal ischemia duringaortic cross-clamping. We report a case of a 77-year-old man who was found to have an AAA associated with a congenital solitary pelvic kidney. An abdominal aortography dearly showed 2 aberrant renal arteries, one of which originated from the aortic wall just above the aortic bifurcation and the other from the left common iliac artery. At surgery, we found other associated anomalies including malrotation of the gut and a left undescended testis. The surgical procedure consisted of an aneurysmorrhaphy followed by a tube graft replacement with therenal arteries being left intact to the distal aortic wall or below. Renal preservation during aortic cross-clamping was achieved by direct perfusion of the upper renal artery with cold lactated Ringer's solution together with topical cooling with ice slush. The patient's postoperative course was uneventful. Urinary output was satisfactory and serum creatinine level remained unchanged throughout his hospital stay. The renal preservation method used in this case was simple and effective.