Patients with concomitant abdominal aortic aneurysms and renal ectopia or fusion abnormalities present a unique technical challenge to the aortic surgeon. The most common of the congenital renal fusion anomalies is the horseshoe kidney, thought to occur in 0.25% of the population. The vast majority of these patients have associated abnormalities in both the number and distribution of renal arteries in addition to anomalies of the renal collecting system. As renal blood supply may be asymmetric, division of the isthmus during aortic exposure can cause irreversible kidney damage. Careful review of preoperative imaging and the identification of aberrant anatomy is thus paramount to ensuring renal preservation. While endovascular aortic aneurysm exclusion is feasible in some patients with renal ectopia/fusion abnormalities, the sacrifice of renal arterial perfusion and contrast-associated nephrotoxicity may preclude an endovascular approach for others. Regardless of approach, with careful preoperative planning, safe aortic aneurysm repair with preservation of renal perfusion is possible for most patients.