From January 1986 to December 1991, 1,059 patients with scoliosis of 20 degrees or more were reviewed. By magnetic resonance imaging, 43 patients were found to have syringomyelia. Thirty-eight patients had Chiari type 1 malformation. Twenty-two of the patients were younger than 10 years. Scoliosis was the only presenting symptom in 32 patients. All patients had some neurologic abnormalities. Fifteen patients had left thoracic curves. The incidence of scoliosis with syringomyelia in our outpatient clinic was 4.0%; 18.4% in boys, and 2.6% in girls. Magnetic resonance imaging should be performed in patients who have abnormal neurologic findings.