An autopsy case of congenital arteriovenous aneurysm of the brain is reported. The patient suffered from hemiplegia for a long time and died of extensive subarachnoid hemorrhage. Postmortem examination revealed large arteriovenous aneurysm, which arised adjacent to the circle of Willis and completely replaced the basal ganglia protruding into the ventricular cavity. Clinical symptoms and cerebral angiogram were characteristic of arteriovenous aneurysm of the brain.