We report the follow-up of a previously published case (Forlodou et al. Neuroradiology 38:595–597, 1996) of carotido-cavernous fistulas (CCFs) in a patient presenting with type IV Ehlers–Danlos syndrome (EDS 4) that were successfully treated twice by an endovascular approach. Initial treatment with a detachable balloon was in 1994 for a right CCF, and, 8 years later, a left CCF was treated by selective transarterial occlusion of the cavernous sinus with coils. Unfortunately, the patient suffered from a spontaneous post-operative intracranial haemorrhage in the left hemisphere and died. Review of the literature, technical considerations for bilateral CCF and complication are discussed.