A 76-year-old woman with abdominal aortic aneurysm coexisting horseshoe kidney and pelvic arteriovenous malformation (AVM) is reported. Operation was performed by a transperitoneal approach via typical median laparotomy. The aneurysm was successfully replaced by a bifurcated prosthetic graft without division of the renal isthmus. Cold lactated Ringer solution was injected into an aberrant renal artery at the time of aortic cross-clamping. This was useful not only to protect the kidney against ischemic injury, but also to know the perfusion area of the vessel. The aberrant renal artery was attached to the prosthetic graft following the aortic proximal anastomosis. Though a part of the asymptomatic AVM, which involved the left ureter, ovary, and uterus, was located anterior to the left iliac arteries, distal anastomoses at the internal and external iliac arteries were done smoothly. No surgical intervention was applied to the AVM in the operation. The post-operative course was uneventful.