Four cases of aneurysms associated with a fenestrated anterior cerebral artery are presented. The combination of aneurysms and fenestration of the anterior cerebral artery is rare, and nine cases including ours have been reported. In all cases fenestrations were found within the distal two-thirds of the horizontal portion of the anterior cerebral artery. It may be presumed that fenestration of the anterior cerebral artery is the remnant of the plexiform anastomosis in the fetal stage, especially between the anterior cerebral artery and the primitive olfactory artery.