The purpose of the study was to further explore the radiological presentations of scoliosis associated with syringomyelia in relation to curve severity. 87 children and adolescents were divided into three groups: Group I (10 degrees <or=Cobb angle<or=30 degrees), Group II (30 degrees <Cobb angle<or=60 degrees), and Group III (Cobb angle >60 degrees). Curves were classified into typical and atypical patterns in the coronal plane and the sagittal profile measured. Cerebellar tonsillar descent or syrinx patterns in relation to curve severity and the frequency of atypical curves were also investigated. The frequency of atypical curve patterns from Group 1 to 2 was 46.2%, 45.2%, and 40.7% respectively. 65.3% patients with typical curve patterns had atypical features in all of the three groups. There was a significant difference of kyphotic angle among the three groups. Both the degree of cerebellar tonsillar descent and syrinx patterns had no correlation with the curve severity or the frequency of atypical curves. These results show that radiographic presentations, including atypical curve patterns, atypical features in typical curve patterns, and a normal to hyperkyphotic thoracic spine may suggest the need for preoperative MRI. There is no evidence to suggest that the degree of cerebellar tonsillar descent and syrinx patterns have an effect on the progress of scoliosis and the frequency of atypical curves.