Perioperative ischemic complications not directly related to surgery require special attention in patients with moyamoya disease; positron emission tomography (H(2) 15O-PET) and single-photon emission computed tomography have been considered indispensable for evaluating pre- and postsurgical cerebral hemodynamics. The clinical records of 14 patients with moyamoya disease who underwent 26 extracranial-intracranial bypass operations were reviewed with special reference to perisurgical complications. One patient developed multiple postoperative ischemic infarctions and died of ischemic brain edema. The history of this patient with prolonged acidosis is analyzed, and the role of metabolic changes induced by H(2) 15O-PET with acetazolamide challenge is reviewed. Seven (77.8%) of nine patients operated on within 48 hours after H(2) 15O-PET with acetazolamide (group 1) developed metabolic acidosis, whereas only three (17.6%) of 17 patients operated on >48 hours (group 2) after the examination had intraoperative pH of <7.35. In group 1, the mean intraoperative pH was 7.328, which was significantly lower than the mean pH of 7.393 (P <.0001) in group 2. After H(2) 15O-PET with acetazolamide challenge, patients must be carefully observed concerning acidosis and volume state. We recommend at least 48 hours between examination and surgery for patients with moyamoya disease so that their conditions can stabilize. Furthermore, special care should be taken to avoid additional perioperative risk factors such as hypotension, hypocapnia, hypercapnia, and hypovolemia.