Spontaneous vertebral arteriovenous fistulae (AVF) associated with neurofibromatosis Type 1 (NF-1) is a rare occurrence in the previous reports. We presented two female NF-1 patients with a diagnosis of cervical vertebral AVF treated with endovascular approach. The fistula in the first case was successfully obliterated by feeder occlusion; whereas, for the second one, new fistulae were formed following the primary fistula closure. Anatomic and hemodynamic changes in combination with dysplastic vascular condition in NF-1 may contribute to fistulae formation de novo. The present case points out a rare challenge encountered in the treatment of vertebral arteriovenous fistula complicated by previous fistula obliteration in NF-1.