Dural arteriovenous fistula (AVF) presenting with subdural hematoma is relatively rare. We report a case of dural AVF presenting as acute subdural hematoma (ASDH) and provide a review of the literature. A 56-year-old man presented with disturbance of consciousness. Computed tomography demonstrated a right ASDH and a small right occipital subcortical hematoma. Cerebral angiography showed a dural AVF on the occipital convexity draining into the cortical veins. Emergent endovascular embolization was immediately performed and the shunt flow disappeared. Hematoma removal and external decompression were safely conducted. Combined therapy successfully recovered the patient's consciousness level. This rare case of dural AVF presenting with ASDH was treated with combined treatments of endovascular and open surgery.