Persistent primitive hypoglossal artery (PPHA) which is a persistent carotid-basilar anastomosis is a rare vascular anomaly. It is usually found incidentally. Fenestrations of cerebral arteries are uncommon. Herein we present a case of PPHA with fenestration of posterior cerebral artery. A 28 year old man was admitted to our hospital with complaints of dizziness and temporary loss of vision. Neurological examination revealed no abnormal findings. The patient was further evaluated with CT and MR angiography. To our knowledge, the case we reported is the first case in the literature with PPHA and fenestration of posterior cerebral artery. The detection of PPHA and associated vascular anomalies and diseases are important before surgical and interventional procedures. CT and MR angiography are much safer diagnostic alternatives in the detection of PPHA and fenestrations. When PPHA is detected, attention should be given to the coexistent posterior circulation anomalies.