Objective: We report a rare case of multiple unruptured cerebral aneurysms associated with persistent primitive hypoglossal artery (PPHA). Case presentation: A 70-year-old female was admitted to our hospital for further examination of left hearing impairment and bilateral tinnitus. 3D-CTA and cerebral angiography showed a large aneurysm at the cavernous segment of the right internal carotid artery (ICA) and a small aneurysm at the top of basilar artery associated with PPHA. The patient underwent balloon-assisted coil embolization of the large aneurysm of the right ICA and rescue stenting for the protruding coil into the right ICA. Postoperative course was uneventful. Conclusion: This is the first report of a large unruptured aneurysm at the cavernous segment of the ICA in multiple cerebral aneurysms associated with PPHA.