Arteriovenous malformation (AVM) of the brain is one of the important pathologic conditions which cause intracerebral or subarachnoid hemorrhage, epilepsy, or chronic cerebral ischemia. The spontaneous regression of cerebral AVM is reported to be very rare and more likely to occur when the AVM is small, is accompanied by hemorrhage, and has fewer arterial feeders. We report a case of right occipital AVM which at follow-up angiography performed four years later showed near-complete spontaneous regression.