Moyamoya disease is a rare progressive cerebrovascular occlusive disease that predominantly occurs in countries in Northeast Asia. We report a 54-year-old male patient with moyamoya and coronary artery disease, on whom urgent off-pump coronary artery bypass grafting was performed after cardiopulmonary reanimation due to ventricular fibrillation. This patient had left main trunk disease, a Pudenz peritoneal catheter (Medtronic, Inc., Minneapolis, MN, USA) for liquor drainage, bilateral internal carotid artery occlusion, diffuse peripheral atherosclerotic artery disease, and chronic renal insufficiency. We prefer off-pump surgery to avoid the risk of intraoperative hypotension and minimize the risk of perioperative cerebral ischemic complications.