The presence of horseshoe kidney in conjunction with abdominal aortic disease significantly increases the technical difficulty of aortic reconstruction. Preservation of the renal blood supply and collecting system during the surgical procedure is the goal of operative management. The pertinent issues that remain unresolved include the need for specific preoperative studies, the optimal operative approach and the safety of isthmus division. From 1979 to 1994 eight patients with horseshoe kidney underwent operative intervention for aortic disease. Five men and three women who had a mean age of 66 years underwent seven reconstructions for aneurysmal disease and one for aortoiliac occlusive disease. All operations were elective and the transperitoneal approach was used in all cases. In the patients with aneurysmal disease the mean maximal aortic diameter was 7.3 cm. The mean preoperative serum creatinine value was 1.1 mg/dl. Preoperative identification of horseshoe kidney was accomplished in all seven patients with aneurysmal disease but not in the patient with occlusive disease. The anomaly was correctly identified by CT scan in seven of seven (100%) patients, arteriography in two of eight (25%) patients, ultrasonography in two of seven (29%) patients, and renal scan in one patient. In the three patients who underwent intravenous pyelography (IVP) the caliceal system was demonstrated to be completely separate from the isthmus. Renal artery anomalies were present in six (75%) patients; in three (50%) these anomalies could be not visualized on the preoperative arteriogram. Renal revascularization was accomplished by a variety of techniques, including reimplantation of multiple (one patient) and single (four patients) renal arteries and branch grafting to an individual renal artery (one patient).(ABSTRACT TRUNCATED AT 250 WORDS)