The coexistence of an abdominal aortic aneurysm and a congenital pelvic kidney is extremely rare. We present a 66-year-old male with an inflammatory aneurysm and an aberrant origin of the superior mesenteric artery. The inflammatory infrarenal abdominal aortic aneurysm with a congenital left pelvic kidney was successfully treated with endovascular repair. Coverage of one out of the two renal ectopic arteries was performed, without clinical evidence of renal function impairment.
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