We are reporting three cases of spontaneous direct carotid cavernous fistulas (CCFs) associated with Ehlers-Danlos syndrome (EDS) out of a series of 147 direct CCFs. The internal carotid artery could be preserved in only one case. Two patients had severe bilateral irregularities of caliber of both internal carotid arteries. Two patients had recurrence of their symptoms after a first balloon was detached in the cavernous sinus (CS) necessitating a second treatment. Two patients died several months later from complications associated with their disease. The treatment of direct CCFs associated with Ehlers-Danlos syndrome is more difficult and more risky than most direct CCFs. The permanent occlusion of the ICA, which may be difficult to avoid, increases the risks of development of aneurysm on the contralateral side.