Dural arteriovenous fistula of the sphenobasilar sinus is a true but rare lesion that connects the meningeal arteries from both the external and internal carotid arteries to the superficial middle cerebral vein (SMCV) and dural sinus. It must be distinguished from other dural arteriovenous fistulas (DAVFs) of the middle cranial fossa, such as cavernous DAVFs and sphenoparietal sinus DAVF, because of differences in the treatment and outcome between these DAVFs. Two patients with sphenobasilar sinus DAVFs reported in the literature have been identified, but they did not simultaneously harbor intracranial meningiomas. To the best of the authors' knowledge, the patient described here is the first case that concomitantly harbors a sphenobasilar sinus DAVF and intracranial meningioma. A 42-year-old man presented with acute subarachnoid hemorrhage. Angiography demonstrated a DAVF of the sphenobasilar sinus with a giant venous aneurysm of the SMCV. After transarterial embolization, the fistula was successfully obliterated and the giant venous aneurysm was resected microsurgically. A fortuitous small meningioma at the anterior clinoid was found and removed during the operation. The patient recovered excellently and resumed his normal activities. The relevant literature is reviewed and discussed.