A 59-year-old male presented with generalized seizure. The patient had not been aware of any traumatic head injuries or preceding infection, and had no contributory medical history. On admission, he was alert and well oriented, without neurological impairment or headache. He was afebrile and blood examination showed no abnormal findings. Computed tomography revealed an irregular intracerebral hematoma, 3 × 1.5 cm in diameter, in the left rectal gyrus. Cerebral angiography showed an arteriovenous fistula (AVF) in the anterior cranial fossa supplied only by the persistent primitive olfactory artery (PPOA) originating from the anterior cerebral artery, forming a shunt to an ascending cortical vein, and drained by the superior sagittal sinus. The patient underwent endovascular obliteration of the AVF via the transarterial route. Immediately after successful isolation, angiography showed that the bilateral anterior ethmoidal arteries supplied the AVF. The feeding branches from the left anterior ethmoidal artery were completely occluded via the ophthalmic artery, but introduction of the catheter into the right ophthalmic artery markedly decreased the stump pressure. Follow-up angiography performed at 3 and 8 weeks following embolization showed spontaneous resolution of the residual AVF without findings of recanalization or new abnormal channels. AVF arising in the anterior cranial fossa may be associated with an unusual pattern of the blood supply when including the PPOA.