A 57-year-old male with a past history of bilateral pulmonary arteriovenous fistulas (PAVFs) experienced a sudden onset of headache and gait disturbance. There was a family history of PAVF and recurrent epistaxis. He had diffuse telangiectasia of the tongue, and hereditary hemorrhagic telangiectasia was diagnosed. Neuroimaging revealed a brain abscess in the right cerebellar hemisphere, which was successfully aspirated under ultrasound guidance. The PAVFs were resected afterwards in two-staged operation. No recurrence of the abscess has been observed. Cerebral abscesses complicated by PAVF are usually supratentorial. Complete eradication of PAVF is essential because the brain abscess will sometimes recur if the PAVF is left untreated.