<h4>Background</h4>A cervical arteriovenous fistula (AVF) in neurofibromatosis type I (NF-1) is uncommon, and it brings challenges and difficulty in treatment.<h4>Case presentation</h4>A 39-year-old woman was diagnosed with an NF-1-associated spontaneous vertebral artery-internal jugular vein-spinal vein fistula. The gene discussed is NF1; the disease is neurofibromatosis type 1.