We describe a patient with a rare association of a parietal arteriovenous malformation (AVM) and an ipsilateral persistent primitive hypoglossal artery (PPHA). A 27-year-old woman was treated by surgical removal, followed by gamma-knife radiosurgery. Only seven cases of intracranial AVM associated with PPHA have been reported in the literature. Although AVM associated with persistent carotid-basilar anastomosis has no distinguishing features compared with ordinary AVM, early recognition of the association is of significance to minimize neurological deficits during diagnostic angiography, interventional radiology (embolization) and surgery. The present report adds to the growing literature regarding AVMs associated with carotid-basilar anastomoses.