STUDY DESIGN: Case report and literature review. OBJECTIVES: To review the English literature pertaining to spondyloptosis of the cervical spine in patients with Von Recklinghausen's disease and to present as an illustrative example the case of a 41-year-old woman with a spondyloptotic kyphotic curve of the spine at C5-C7 of more than 110 degrees. SUMMARY OF BACKGROUND DATA: Involvement of the cervical spine in neurofibromatosis has only rarely been documented, although the spine is the part of the skeleton mostly affected in this hereditary disease. Only a few cases with a cervical kyphotic curve exceeding 90 degrees or with cervical spondyloptosis have been reported until now. METHODS: A literature and chart review was conducted. The patient was first treated conservatively, but over time, the spontaneous neck pain increased to an intolerable level and progressive neurologic deficits developed in the four limbs. For these reasons, surgical intervention was undertaken, according to suggestions from the literature. RESULTS: Postoperative imaging showed improved realignment of the cervical spine with a residual kyphos of 30 degrees. At later follow-up stable bony fusion was obtained in the lower cervical spine. CONCLUSIONS: A successful one-stage anterior and posterior correction and fusion-stabilization procedure was performed with extension from the occiput to T1.