Abstract We report a case of a dural arteriovenous fistula (DAVF) at the tentorium cerebelli, which presented progressive myelopathy. A 68-year-old man with neurological deterioration of the cervical myelopathy visited our hospital. T2 weighted magnetic resonance (MR) imaging showed high signal area and edema from the medulla to the upper thoracic spinal cord with flow voids on the dorsal surface of the cord. Angiography showed right tentorial DAVF, which was supplied by the right meningohypophyseal trunk, the middle meningeal artery, the accessory meningeal artery, and was drained into the posterior spinal veins. The patient underwent right retrosigmoid suboccipital craniotomy, then disruption of the fistula was performed by using micro Doppler sonography following endovascular obliteration of the main feeders. Postoperative angiography showed complete obliteration of the fistula. His daily functioning gradually improved up to 6 months after the surgery. Tentorial DAVFs with clinical manifestation of myelopathy are rare. Considering its aggressive nature, early surgical treatment could be necessary. (Received: November 17, 2010, Accepted: December 18, 2010).