We report a rare case of persistent primitive hypoglossal artery with complete agenesis of bilateral vertebral arteries. MRI showed diffuse cerebellar, posterior parietal and occipital atrophy with only mild atrophy of brain supplied by anterior circulation. On further evaluation with MR Angiography, right sided persistent primitive hypoglossal artery was seen arising from the internal carotid artery and continuing to form basilar artery with agenesis of bilateral vertebral and posterior communicating arteries. Posterior cerebral and cerebellar atrophy seen in this patient can be attributed to the persistent primitive hypoglossal artery being the sole blood supply with no communication between anterior and posterior circulation with absence of bilateral posterior communicating arteries.