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Carotid-cavernous fistula was one of the first intracranial vascular lesions to be recognized. This paper focuses on the historical progression of our understanding of the condition and its symptomatology—from the initial hypothesis of ophthalmic artery aneurysm as the cause of pulsating exophthalmos to the recognition and acceptance of fistulas between the carotid arterial system and cavernous sinus as the true etiology. The authors also discuss the advancements in treatment from Benjamin Travers' early common carotid ligation and wooden compression methods to today's endovascular approaches.