In a cohort of 310 consecutive congenital scoliosis patients, a retrospective analysis of abnormalities in ten organ systems was performed. Overall, intraspinal defects were observed in 43% of patients and defects in other organ systems were found in 77% of patients. Urogenital anomalies occurred in 39% of patients. Cardiac anomalies occurred in 54% of patients, a rate more than two times higher than previously reported. The association between defects in various organ systems was also elucidated.