The natural history, associated anomalies, and response to operative and nonoperative treatment were reviewed in 102 patients with neurofibromatosis and spine deformity. Eighty patients were found to have curvatures associated with dystrophic changes in the vertebrae and ribs. The presence of dystrophic changes such as rib penciling, spindling of the transverse processes, vertebral scalloping, severe apical vertebral rotation, foraminal enlargement, and adjacent soft-tissue neurofibromas was found to be highly significant in prognosis and management. Brace treatment of dystrophic curves was unsuccessful. Posterior fusion, with or without internal fixation, was the procedure of choice for problems due purely to scoliosis. Patients with dystrophic kyphoscoliosis required both anterior and posterior fusion to achieve stability. Sixteen patients had compression of the spinal cord or cauda equina.