A case of spontaneously occurring direct carotid-cavernous fistula complicated by choroidal detachment is presented. After thrombosis of the superior ophthalmic vein, paradoxical worsening of the clinical manifestations, recurrence of the choroidal detachment and subsequent resolution of symptoms were observed. We concluded that carotid-cavernous fistula should be kept in mind in the etiological evaluation of choroidal detachment.