Summary: A retrospective review of 25 patients with scoliosis secondary to syringomyelia was conducted. All patients had large syrinxes, but only 10 patients had abnormal neurologic findings, suggesting that subtle neurologic findings in patients with scoliosis should not be underestimated and supporting liberal use of magnetic resonance imaging (MRI). The results of three approaches to the care of these patients were reviewed: passive observation, bracing, and spinal fusion. Curves progressed ≥5° in patients aged <10 years who were observed, but in children aged >10 years, curves progressed ≥10°. Curves responded well to bracing, but correction was not maintained out of brace for patients with progressive and/or severe curves. Good correction was achieved safely during operation.