Abstract A case of spontaneous carotid-cavernous fistula associated with Ehlers-Danlos syndrome Type IV was treated successfully by transarterial balloon embolization. To minimize the stress to the arterial wall, we detached three silastic balloons with low-attachment force in the cavernous sinus with the aid of a proximal balloon to control the high flow of the fistula. To achieve complete obliteration, we had to inflate the third balloon to dimensions that occluded the cavernous portion of the internal carotid artery. Patients with Ehlers-Danlos syndrome Type IV carry a high risk when undergoing any diagnostic or therapeutic endovascular procedure due to the fragility of their blood vessels. Recognition of this entity is important and special attention should be paid to its pathophysiology for a successful treatment.