We successfully treated a case of a 65-year-old female with an abdominal aortic aneurysm coexisting with an atypical coarctation of thoracoabdominal aorta and celiac axis and superior mesenteric artery occlusion. A dilated inferior mesenteric artery was supplying the celiac artery and superior mesenteric artery regions. The patient also had mitral regurgitation. After a mitral valve plasty, we repaired the abdominal aortic aneurysm and the atypical coarctation of the thoracoabdominal aorta using partial extracorporeal circulation, segmental clamping, and a selective perfusion of both the bilateral renal artery and dilated inferior mesenteric artery. The patient had an uneventful hospital course and remains well.