The purpose of this study was evaluate the operative procedure and outcome of abdominal aortic aneurysm (AAA) patients with aortocaval fistula (ACF) and iliac vein fistula. From 1982 through 2004, we experienced five AAA patients associated with spontaneous aortocaval and aortoiliac venous fistula who underwent repair of AAA. Three patients were in hypovolemic shock, including one patient with cardiopulmonary arrest on admission who required cardiopulmonary resuscitation before surgery. These three ACF patients with hypovolemic shock underwent emergency operation and two patients with stable hemodynamic state underwent urgent operation. One of two ACF patients with stable condition was associated with unstable angina. One AAA patient with ACF-complicated angina underwent AAA repair with coronary artery bypass grafting; the remaining four patients underwent 3 bifurcated graft and 1 tube graft implantation. All surgical treatment of the fistula included direct closure within the aorta under digital compression in four patients and inferior vena cava clamp in one. The mortality rate was 25%. One ACF patient with retroperitoneal hematoma died of bleeding. Survival for ACF depends on early diagnosis and prompt surgical repair. Aortocaval fistula complicated with a rupture of aneurysm into retroperitoneal space had a severe fatal prognosis compared with uncomplicated ACF.