Anti-phospholipid antibody syndrome(APS) is characterized by the presence of antibodies to phospholipids and various symptoms including those derived from thrombosis. APS has been shown to be frequently accompanied by multiple and recurrent cerebral ischemic lesions, suggesting that APS may play a critical role in developing cerebral ischemia of unknown etiology. Here, we present a case with an unruptured cerebral aneurysm accompanied by a severe stenosis of the middle cerebral artery as a manifestation of APS. The aneurysm was successfully treated surgically employing the following precaution to prevent potential damage on the fragile ischemic brain: preoperative administration of anticonvulsant, intermittent brain retraction, intraoperative SEP monitoring, and postoperative administration of anticoagulant. Our experience suggests that unruptured cerebral aneurysms accompanied by APS could be safely treated by strict paraoperative management.