The authors report on a young woman with Ehlers-Danlos syndrome (EDS) Type IV in whom a spontaneous direct carotid-cavernous fistula (CCF) was treated by transvenous occlusion with regular and fiber-coated Guglielmi detachable coils. To the authors' knowledge, this is the first time this approach has been used in a patient with EDS. The different treatment options are discussed, and the literature on endovascular treatment of direct CCFs in EDS is reviewed.