Abstract： Carotid cavernous fistula usually presents with ocular symptoms as the first clinical manifestation.Due to its variable phenotype,patients are often misdiagnosed as other ocular diseases which can lead to missed diagnosis and delayed treatment.Therefore,we reviewed the literature on carotid cavernous fistula to help understand the ocular charac-teristics,classification diagnosis,possible treatment mode and expected outcome of carotid cavernous fistula.