The treatment of a carotid cavernous fistula (CCF) depends on the severity of the clinical symptoms, its angiographic characteristics, and the risk it presents for intracranial hemorrhage. In most instances, endovascular treatment is preferred.High-flow direct CCFs usually are traumatic or are caused by rupture of a cavernous aneurysm into the sinus, but a small percentage can be spontaneous. They usually present with sudden development of a clinical triad: exophthalmos, bruit, and conjunctival chemosis. All direct CCFs should receive treatment, because they carry a high probability of intracranial hemorrhage or neurologic deterioration.Low-flow indirect or dural CCFs, either incidental or with minimal symptoms, are not associated with significant risk of intracranial hemorrhage. The accepted practice is to treat ocular symptoms conservatively with medical management or manual carotid compression. If the patient cannot tolerate the symptoms, or if signs of ocular morbidity occur, endovascular treatment is offered.The first treatment option should be endovascular embolization with a combination of detachable balloons, coils, stents, or liquid embolic agents. The procedure can be performed from either an arterial or venous approach. Use of these materials and techniques can yield a high cure rate with minimal complications.If the patient is not amenable to embolization or if the embolization fails, then surgery (surgical ligation of the internal carotid artery or packing of the cavernous sinus) should be offered.Stereotactic radiosurgery may be an elective treatment for low-flow CCFs, but it has no role in the treatment of high-flow CCFs.