The patient was a 10-year-old boy. He had a sudden onset of generalized tonic-clonic convulsion. On admission, he presented with mild disturbance of consciousness and right hemiparesis. The electroencephalogram showed diffuse spike and slow wave complex. Three days later, magnetic resonance imaging (MRI) showed high signal intensity areas in the left cerebellar hemisphere and pons. On cerebral angiograms, coiling of the bilateral carotid arteries, occlusion of the basilar artery at the distal site, and fenestration of the left vertebral artery at the atlanto-axis level were shown. According to these findings, the patient was diagnosed with brainstem and cerebellar infarction. On the follow-up angiograms, recanalization of the basilar artery on the right vertebral angiogram, occlusion of the left vertebral artery at the distal site of the posterior inferior cerebellar artery origin, and occlusion of one of the duplicated vertebral arteries were recognized. Fenestration of the vertebral artery is presented in about 1-2% at angiography and autopsy and the clinical significance is controversial. In this case, the fenestration may have played a role as an embolic source because there was no probable cause of the cerebral infarction, and the vascular occlusion and recanalization occurred near the distal site of the fenestration. These findings suggest the clinical significance of vertebral artery fenestration as an embolic source.