Three kinds of carotid-basilar anastomosis have been found, they were named as persistent primitive trigeminal artery, persistent primitive acoustic (or otic) artery and persistent primitive hypoglossal artery, among them, the persistent primitive hypoglossal artery is by far the least common type of the internal carotid-basilar anastomosis which have been demonstrated in human postembryonic stages. Persistent primitive hypoglossal artery is usually found incidentally. This vascular anomaly, however, have been reported to be associated with multiple congenital anomalies, such as unilateral or bilateral vertebral artery hypoplasia, abscence of the posterior communicating attery, hypoplasia of the proximal portion of the posterior cerebral artery, aneurysm of the circle of Willis, the origin of both pericallosal arteries from a single anterior cerebral artery, retarded mental development dating from infancy, carotid-cavernous fistula formation etc. A case of persistent primitive hypoglossal artery in an young Korean male was reported with brief review of the lrterature.