A case of an iatrogenic direct carotid cavernous fistula in a patient with a history of Ehlers-Danlos syndrome and multiple aneurysms is reported. The fistula developed after unsuccessful surgical thrombectomy and revision of an occluded interposition graft inserted to treat a right internal carotid artery aneurysm. Direct puncture of the right internal carotid artery at the level of the skull base was performed to close the fistula. This case shows that direct puncture at the highest cervical segment of the internal carotid artery is another option for treatment of a direct carotid cavernous fistula when a standard transarterial or transvenous approach is not feasible.