A case of traumatic, direct, carotid cavernous fistula (CCF) associated with Ehlers - Danlos syndrome (EDS) Type IV is reported along with a review of the literature. Excluding the present case, three similar cases associated with EDSTypeJV have already been reported by Gerard M. Debrun et Al(l). Despite the risks associated with endovascular manipulation, the fistula was successfully closed by intravascular embolisation but the patient expired a few days later because of underlying disease-associated vascular and visceral complications.