The primitive hypoglossal artery may rarely persist into adult life. The artery arises from the internal carotid artery in the base of the neck, enters the cranial cavity through the anterior condylar canal and terminates by becoming the basilar artery. The vertebral arteries are hypoplastic. The authors present a case where the hypoglossal artery arose from a stenosed internal carotid artery. Such a combination of lesions is of grave significance as the basilar territory becomes compromised as well as the ipsilateral cerebral hemisphere.