To investigate the prevalence of and to identify independent predictors associated with scoliosis in patients with multiple hereditary exostoses (MHE).Fifty patients with MHE were clinically examined, and the diagnosis of scoliosis was made based on radiographs. To classify disease severity, three classes based on the presence of deformities and functional limitations were defined. Significant independent predictors of scoliosis in MHE were statistically analyzed.Scoliosis was present in 36 patients (MHE-scoliosis) (72 %). In the MHE-scoliosis group, the mean primary curve was 15.3° ± 5.7° (range 10°-34°) and the mean minor curve was 10.6° ± 7° (range 6°-32°). Left curve was predominant (72 %), and the apex was located in the thoracolumbar or lumbar spine in 64 % of patients. Univariable and multivariable analyses confirmed that MHE severity was a significant predictor of moderate scoliosis (≥20°).Our study confirmed that scoliosis is a common feature of MHE and disease severity is a predictor of moderate scoliosis (≥20°).