We describe a patient with an aneurysm arising at a persistent primitive trigeminal artery ruptured to form a carotid-cavernous fistula. Coil occlusion of the carotid cavernous sinus fistula resulted in flow stasis of the persistent primitive trigeminal artery and resolution of symptoms. A ruptured aneurysm of the persistent primitive trigeminal artery can be associated with a carotid cavernous sinus fistula. Coil occlusion of the cavernous sinus and the PPTA is a safe and effective technique to treat this disease.