An autopsy case of persistent primitive hypoglossal artery (PPHA) with multiple cerebral aneurysms is reported. A 54-year-old man with subarachnoid hemorrhage was admitted to Kuwana Hospital three days after the onset. The patient was stuporous and had stiffness of the neck. A computed tomogram showed hematoma in the interhemispheric fissure, subarachnoid hemorrhage in the basal cisterns and bilateral Sylvian fissures, and maxked dilatation of ventricles. Cerebral angiogram revealed the left PPHA and multiple aneurysms at the right anterior cerebral artery (A 2) (ruptured), anterior communicating artery, left anterior cerebral artery (A 1), left internal carotid-anterior choroidal artery junction, right internal carotid artery (C 1), and right middle cerebral artery. Neck clipping of the ruptured aneurysm and ventricular drainage were performed on the day of admission. Eight days after admission he died of rupture of the residual aneurysm. In pathological study, the PPHA was originated from the extracranial portion of the left internal carotid artery, 2 cm distal from the cervical carotid bifurcation, entered the intracranial space through the hypoglossal foramen, and turned into the basilar artery. There were six aneurysms which were shown on cerebral angiogram and another aneurysm on the left anterior inferior cerebellar artery. Microscopic examination revealed atherosclerotic change of the PPHA, true aneurysmal changes of the seven aneurysms and defect of tunica media (Forbus' medial gap) at all of the arterial bifurcations without early aneurysmal changes.