The persistent primitive hypoglossal artery (PPHA) is the second most common persistent carotid-vertebrobasilar anastomosis, with an incidence of 0.027-0.26%. PPHAs change the hemodynamics of the carotid and vertebrobasilar system and may be associated with intracranial vascular anomalies, but basilar bifurcation aneurysms were rarely reported. We describe the first case of a PPHA as the sole supply to the brain associated with a basilar bifurcation aneurysm and review the literature. We reported a 34-year-old woman who presented with subarachnoid hemorrhage due to a ruptured basilar bifurcation aneurysm. Digital subtraction arteriogram revealed a right PPHA as the sole supply to the brain. The aneurysm was successfully clipped under intraoperative neurophysiology.