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Horseshoe kidney is a rare congenital anomaly that can create various technical problems during surgery for repair of abdominal aortic aneurysm. The diagnosis of this anomaly should be confirmed preoperatively in order to plan surgical strategy. Nowadays, in more than 90% of all cases, ultrasonography, contrast computerized tomography, urography, and angiography are the best instrumental methods of detecting this anomaly in association with abdominal aortic aneurysm. The transperitoneal approach assures the best exposure of the kidney, the ureters, the aneurysm, and both iliac vessels, but the renal isthmus can pose a problem in reimplanting aberrant renal arteries. When it is known preoperatively that renal revascularization should be performed, the left extraperitoneal approach is a better choice. In any event, the coexistence of horseshoe kidney and abdominal aortic aneurysm does not preclude the treatment of the latter. In elective surgery of abdominal aortic aneurysm, the morbidity and mortality rates in the presence of horseshoe kidney are much the same as those in the presence of normal kidneys. The best results in this kind of surgery are obtained by adapting one's surgical technique to each anatomical variant that is encountered.