Middle mesenteric artery has been described in 1923. We report the observation of a patient with an abdominal aortic aneurysm who had this rare artery arising from the anterior wall of the aneurysmal sac. His inferior mesenteric artery was occluded at its origin from the aorta and the middle and the distal colon was vascularized only by the middle mesenteric artery. Occlusion of this artery would have been necessary before endovascular repair of the aneurysm. We were concerned about the risk of colic ischemia after the occlusion of the middle mesenteric artery, so we abandoned this approach and operated on the patient via a laparotomy. Based on a case report, we here report a literature overview on the repair of abdominal aortic aneurysm in the presence of a middle mesenteric artery.