BackgroundA cervical arteriovenous fistula (AVF) in neurofibromatosis type I (NF-1) is uncommon, and it brings challenges and difficulty in treatment.Case PresentationA 39-year-old woman was diagnosed with an NF-1-associated spontaneous vertebral artery-internal jugular vein-spinal vein fistula. The fistula was placed by coil embolization. Postoperative examination showed that the fistula closure was satisfied, and the patient's abnormal clinical manifestation disappeared without any complications after 24 months of interventional embolization. As per the literature, interventional embolization is currently the main treatment method, and it has the distinguishing features of less trauma, quick recovery, and a good prognosis.ConclusionNF-1 associated with a spontaneous arteriovenous fistula is rare in clinical practice, which carries significant challenges in treatment, but can be effectively treated using endovascular embolism. Endovascular embolism could be the potential choice of treatment in NF-1 associated with AVF.