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Carotid-cavernous fistula (CCF) associated with persistent trigeminal artery (PTA) is a rare but important clinical entity. We present a case treated by microcoil embolization with preservation of internal carotid, PTA, and hasilar artery flow following embolization. A 62-year-old female developed pulsatile tinnitus followed by left eye proptosis and diplopia. Examination revealed a cranial nerve VI palsy and an objective bruit over the left orbit. Angiographic evaluation revealed a carotid cavernous fistula originating from a persistent trigeminal artery. Placement of a detachable balloon across the fistula site while preserving the PTA proved impossible, and the fistula was treated with microcoils following placement of a microcatheter across the fistula into the cavernous sinus. Complete closure of the fistula was followed by resolution of the patient's symptoms. Preservation of all major vessels including the PTA was accomplished through the use of coil embolization. Careful evaluation of the angiogram is necessary to identify PTA associated with a CCF. Previous reports have described treatment of CCF with PTA by surgical or balloon ocolusion, some involving sacrifice of the PTA. Examination of the relevant embryology and anatomy reveals, however, that occlusion of the PTA must be approached with caution due to potential supply to the posterior circulation.