The authors analysed an unusual case of dural arteriovenous fistula (DAVF) of the transverse-sigmoid sinus causing trigeminal neuralgia is presented. Although progression to almost continuous facial pain has been reported, symptoms may be indistinguishable from typical trigeminal neuralgia. The patient had a 6-year history of right-sided trigeminal neuralgia initially well controlled by medical management. He was referred for surgical management after 10 months of progressively worsening of symptoms. At the time of consultation, the patient complained of pulsatile tinnitus in the right ear. Computed tomography imaging and angiography demonstrated a DAVF involving the right transverse-sigmoid sinus junction with retrograde venous drainage. Surgical resection of the DAVF provided both angiographic cure and complete relief of all symptoms. The authors discuss the pathophysiology of trigeminal neuralgia in patients with a DAVF.