It is well known that neurofibromatosis is associated with various types of arteriovenous fistulas (AVF), but association with an occipital AVF has not been reported. We are reporting this case which was successfully treated with a combination of embolization and surgery. Superselective angiography defines the anatomic details of the fistula and intraoperative digital subtraction angiography (DSA) facilitates the surgery to cure the fistula. It is important to identify the best therapeutic options using angiographic information to treat a complex arteriovenous fistula.