Spontaneous carotid-cavernous fistula is a very rare but serious complication that can compromise functional or vital prognosis; clinical diagnosis is suggested by ophthalmic and orbital signs, which must be actively sought. Daily monitoring using transcranial Doppler can enable early detection and indicate the need for further investigations. The visualisation of direct or indirect signs on a brain CT scan should lead to the performance of diagnostic and therapeutic cerebral angiography. The role of interventional neuroradiology appears to be indisputable as a first-line treatment. The indications for surgical carotid sacrifice are currently limited to cases where endovascular techniques have failed.
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