Dural ateriovenous fistula (DAVF) at the craniocervical junction is rare. We report a patient presenting with brainstem dysfunction as an uncommon onset. Brainstem lesion was suggested by magnetic resonance image study. Angiogram revealed a DAVF at a high cervical segment supplied by the meningeal branch of the right vertebral artery, with ascending and descending venous drainage. Complete obliteration of the fistula was achieved via transarterial Onyx embolization. Clinical cure was achieved in the follow-up period; meanwhile, imaging abnormalities of this case disappeared. Accordingly, we hypothesize that a brainstem lesion of this case was caused by craniocervical DAVF, which induced venous hypertension. Thus, venous drainage patterns should be paid attention to because they are important for diagnosis and theraputic strategy.