We defined the natural history of scoliosis in congenital heart disease (CHD) with respect to the risk of progression and indications for treatment. We reviewed radiographs of 48 children with CHD and scoliosis. Thirty-seven had developmental scoliosis (77%), and 11 (23%) had congenital scoliosis. Children with congenital scoliosis can be managed in the same way as children without CHD. Developmental curves < 30° in children aged > 10 years showed no progression. Developmental curves > 30° with onset in children aged < 10 years and progression > 9° per year require treatment.