Objective. The Purpose of this study was to evaluate technical problems and results of surgical treatment of aortic disease associated with major renal anomalies. Study design. Renal anomalies observed during aortic procedures performed over a 19-year period were reviewed. Type of renal anomaly, diagnostic procedures, Preoperative renal function, technique of vascular reconstruction, and management of the anomalous kidney were considered and correlated with perioperative and long-term results. Results. Eighteen patients (1.1%) with major renal abnormalities were found in our experience with more than 1650 aortic procedures. Of these, 10 involved horseshoe kidneys and 8 ectopic kidneys (3 pelvic, 3 lumbar, and 2 crossed ectopias). All elective cases were evaluated with computed tomography, urography, and angiography. Aortic reconstruction was performed in 17 patients for aortic aneurysm (1 urgent repair for rupture) and in 1 patient for obstructive disease. Surgical access was anterior transperitoneal in 16 patients and retropeyitoneal/thoracoabdominal in 2. In 8 patients, reimplantation of 1 or both renal arteries was necessary. Division of the renal isthmus was necessary in 3 patients with horseshoe kidney. The perioperative mortality rate was 0%. Renal function was normal at discharge in all patients. At long-term follow-up (median, 5 years; range, 1 to 19 years) neither renal nor aortic complications were detected. In 15 of 17 patients, the aneurysm was confined to the abdominal aorta. Conclusion. Although infrequent, renal anomalies encountered during aortic surgery can be effectively treated with accurate diagnosis and preoperative planning.