We report a caroticocavernous fistula (CCF) treated by an endovascular procedure in a young woman with Ehlers-Danlos syndrome type IV, with severe bilateral carotid and vertebral artery dysplasia. The CCF, which appeared after minor trauma, was successfully occluded by a detachable balloon introduced into the venous side by an arterial approach. Six previously published cases are reviewed, five successfully treated. The difficulties and risks of the endovascular procedure due to the vascular changes, are emphasised. The possibility of the venous approach is discussed.