A case of persistent primitive trigeminal artery with vertigo as the initial symptom is reported. The case was a 41-year-old female. Since 2002, the patient had repeated episodes of vertigo. In 2004, the patient experienced a loss of consciousness. No lesion causing dizziness was found by CT scan of the brain, MRI of the brain, ECG, brain wave examination, and a balance function test at the Department of Otolaryngology. Persistent primitive trigeminal artery and vertebrobasilar hypoplasia were suspected by MRA and the diagnosis was confirmed by angiography. It is advisable to consider MRA and angiography for vertigo or dizziness of unknown cause.