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We present a rare case of a patient with aortoiliac occlusive disease on the background of type A crossed renal ectopia, for whom open surgical intervention was required. Aortic exposure in patients with concomitant crossed renal ectopia can present technical challenges to the vascular surgeon. The knowledge of variations in the ectopic renal blood supply is of paramount importance when performing surgery to treat this condition and affects the choice of surgical exposure. We present and discuss the operative details of our patient and outline an approach to this subset of patients.