Background: Trigeminal neuralgia (TN) is mostly caused by vascular compression of the nerve's root entry zone due to an ectatic artery. Rarer causes include compression from tumors, vascular malformations or multiple sclerosis plaques. Developmental venous anomalies (DVAs) are benign, aberrantly appearing venous structures that drain normal cerebral tissue. DVAs are a rare etiology of TN. The management of TN caused by a DVA is controversial as disruption of the DVA can be catastrophic. Methods: We report a case of a young man with severe medically refractory TN related to a brachium pontis DVA who was successfully treated by gamma knife stereotactic radiosurgery (GKSR) to the trigeminal nerve. Results: Within 2 weeks of GKSR, the patient reported experiencing 60% pain relief; 5 years postoperatively, he remains completely pain free with some mild sensory loss in the V2 and V3 areas. Conclusions: GKSR has an established role in the management of TN. This is the first reported case of using GKSR to treat TN caused by a DVA. In the setting of a DVA, GKSR should be an initial consideration for TN therapy after medical failure because of the high surgical risk related to disrupting the DVA. © 2015 S. Karger AG, Basel.