Ectopic major renal arteries are rare but anatomically important because they can complicate aortic surgery and make the operation challenging for vascular surgeons. A 68-year-old male was presented with a 5.5-cm aneurysm of the infrarenal abdominal aorta combined with an ectopic main right renal artery arising from the middle of the aneurysm sac, perfusing a normotopic right kidney. The patient also had small right common iliac artery aneurysm. Open repair was performed with reimplantation of the right renal artery on the aortic tube graft, the right kidney was perfused with cold heparinized lactated Ringer solution during operation. The right common iliac artery aneurysm was wrapped with a polyester band. The patient's postoperative courses were uneventful, with normal renal function during 5 years of follow-up. Preoperative planning is important for achieving optimal results in treating complex aneurysms with ectopic main renal artery.