Spinal dural arteriovenous fistulae (DAVF) affect predominantly levels of the lower thoracic and lumbar segments; only 13 cases have been reported of DAVF at the foramen magnum. We present three surgically treated patients with DAVF at the foramen magnum. In none of our three patients could the site of the arteriovenous fistula be suspected from the clinical presentation. The clinical course varied from acutely developing signs and symptoms to a 10-year history of very slowly progressing symptoms. After neuroradiological diagnosis the patients were operated on direct microsurgical disconnection of the arteriovenous shunt via an enlargement of the foramen magnum and a hemilaminectomy of C1. DAVF at the foramen magnum may thus present with slowly to acutely progressing clinical symptoms and signs. Spinal angiographic examination should include the level of the foramen magnum if standard spinal angiography of thoracic, lumbar, and sacral segments is negative in suspected spinal DAVF since the nidus of the shunt can be situated remote from the level of neurological disorder. DAVF at the foramen magnum can be treated very effectively and with minimal surgical trauma by direct microsurgical disconnection of the shunt. This surgical procedure is indicated if embolization with glue is not possible or is unsuccessful.