Thirteen children with moyamoya disease who had no apparent cerebral infarction or hemorrhage were examined pre- and postoperatively by means of positron emission tomography (PET) to investigate the underlying cerebral circulation and metabolism and the effect of bypass surgery. The preoperative regional cerebral blood flow (rCBF) and mean transit time were significantly decreased and increased, respectively, in the cerebral cortex of these patients compared to control values. The regional cerebral blood volume (rCBV) and the regional oxygen extraction fraction (rOEF) had significantly increased to compensate for the reduced rCBF and perfusion pressure and also to maintain the regional cerebral metabolic rate of oxygen (rCMRO2). In the basal ganglia, rCBV elevation was more prominent than that in the cerebral cortex, although changes in rCBF, rOEF, and rCMRO2 were relatively minor. Postoperative improvements were observed predominantly near the cortex where bypass surgery had been performed and in the basal ganglia. Direct and combined indirect bypass procedures improved cerebral circulation more effectively than single indirect bypass surgery. Although the angiographic findings were not always compatible with the clinical results, the postoperative improvements on PET scans correlated with the disappearance of transient ischemic attacks. In addition to the clinical courses and angiographic findings, PET analysis was indispensable in evaluating the cerebral circulation and metabolism in childhood moyamoya disease.