A persistent primitive olfactory artery (PPOA) is an extremely rare variation of the proximal anterior cerebral artery (ACA). The PPOA is connected to the distal ACA, ethmoidal artery, or both. I describe one patient with a PPOA connected to the middle cerebral artery (MCA). I analyzed the radiological characteristics of this anomalous vessel in this patient, who presented with headache. Computed tomography-angiography revealed an abnormal vessel in the patient, which originated from the distal ACA and ran anteromedially along the olfactory tract; it then made an abrupt turn and became the MCA. A PPOA connected to the MCA has been described in only two patients, including my own, in the English-language literature.