A 16-year-old boy who had been diagnosed previously as Aarskog-Scott syndrome (AAS), referred to our clinic with shoulder asymmetry for 1 year. Results of spine examination showed a 52 degrees right thoracic curve at T3-T11. Surgery was planned, and T1-L1 posterior instrumentation and fusion were performed. After surgery, satisfactory correction was achieved, and during 10 years follow-up, the patient had no complaints. AAS is a X-linked genetic disorder with facial, genital, and skeletal manifestations. Scoliosis is not reported as a typical finding of AAS, and there is no reported case in the English literature. Due to mutation affecting the developing skeleton tissue, spinal deformities may develop. In our case, concave side fusion was seen at the deformity. Although we do not know any specific pattern of the scoliotic deformity of this syndrome, surgical correction of the deformity can be difficult because of the premature fusion at these levels.