We report a successful case of ruptured abdominal aortic aneurysm with horseshoe kidney treated by graft replacement following the insertion of an aortic occlusion balloon catheter. An 80-year-old man, presenting with sudden abdominal pain, was transferred under the diagnosis of ruptured abdominal aneurysm. Enhanced CT showed infrarenal abdominal aortic aneurysm, retroperitoneal hematoma, and horseshoe kidney. As soon as possible, we performed indwelling aortic occlusion balloon catheter via a femoral artery under radiographic guidance. At the time of induction of anesthesia, because of the collapse of hemodynamics, we inflated the balloon to stabilize the patient's blood pressure, and performed the bifurcated graft replacement and inferior mesenteric artery reconstruction with preserved isthmus of horseshoe kidney in safety. The postoperative course was uneventful, and the patient was discharged on the 14th postoperative day without any morbidity.