A 68-year-old male presented with a syncopal attack subsequent to acute myocardial infarction. His ultrasonographic and radiological examination revealed severe left internal carotid artery (ICA) stenosis and the presence of a persistent primitive hypoglossal artery (PPHA) immediately distal to the stenosis. The bilateral anterior and left middle cerebral arteries, and the vertebrobasilar system were opacified via the stenotic ICA. Carotid arterial stenting was selected as the treatment method because the lesion was high and a shunt placement during carotid endarterectomy was considered to be technically difficult. A self-expanding stent was successfully deployed with flow control, and the patient was discharged six days after surgery without any neurological deficit. There are sixteen reported cases including ours of PPHA associated with ICA stenosis presenting with ischemic attacks of the vertebrobasilar system. To the best of our knowledge, the current case is the first report of a cervical ICA stenosis with ipsilateral carotid-basilar anastomosis treated with carotid arterial stenting.