A carotid cavernous fistula is an abnormal communication between the internal carotid artery and the cavernous sinus. Rarely, this communication is associated with a persistent primitive trigeminal artery, with or without a trigeminal artery aneurysm. We report a case of spontaneous carotid-trigeminal cavernous fistula in which the persistent trigeminal artery was shown only on vertebral artery injection. The absence of an associated trigeminal artery aneurysm allowed a transvenous approach for endovascular treatment with coils and complete obliteration of the cavernous fistula.