We presented a case of neurofibromatosis type I (NF-1) associated with an extracranial arteriovenous fistula (AVF) fed by the occipital and vertebral artery. A 20-year-old man was referred to our hospital because of an occipital subcutaneous pulsatile mass. A CT scan showed a huge subcutaneous enhanced mass. Angiography revealed that the occipital AVF was fed by bilateral occipital arteries, the left ascending pharyngeal artery, the left middle cerebral artery, and the left vertebral artery with-abundant communication with the subcutaneous veins. Endovascular treatment by using both coil and glue (Eudragid) embolization via the occipital artery successfully obliterated the AVF. Subsequently surgical operation was performed. Postoperative angiography showed the disappearance of the AVF. Combination of intravascular surgery and surgical operation should be considered as an effective treatment for NF-1 associated with AVF.