When evaluating patients with neurofibromatosis and scoliosis, a careful search for the evidence of dystrophic changes should done, because prognosis and management depend highly on the presence of these changes. Preoperative imaging studies including computed tomography or magnetic resonance imaging are recommended, especially in cases of dystrophic scoliosis, to detect unrecognized intraspinal lesions. The radiographic findings and the response to treatment in patients with nondystrophic scoliosis are similar to those in patients with idiopathic scoliosis, and the same decision-making process for idiopathic scoliosis is applicable. Careful follow-up evaluation is mandatory, however, because of the tendency for extraordinary progression to occur with growth. Brace treatment of the short, angulated, dystrophic curves is ineffective; early and aggressive surgical intervention is strongly recommended. In most cases, dystrophic curvatures are best treated with combined anterior and posterior spinal arthrodesis. The fusion area should be generous, and the entire structural area of the deformity both anteriorly and posteriorly should be fused.