PURPOSE: The purpose of this study was to evaluate the management and outcomes of CDH patients with scoliosis. METHODS: From January 1996 to August 2015, 26 of 380 (7%) CDH patients were diagnosed with scoliosis. Six (23%) were prenatally diagnosed by ultrasound, and 9 (35%) were diagnosed postnatally. The remaining 11 (42%) developed scoliosis after discharge. Mean follow-up was 6.6years. RESULTS: Among the 15 patients with congenital scoliosis, there were 2 (13%) perinatal deaths. Five of the 13 (38%) survivors required orthopedic surgery, and 2 have required bracing. The mean age at initial surgery was 7years. These five children underwent an average of 2.8 (range 1-7) expansions or revisions. All surgical patients required supplemental oxygen at 28days of life, and 1 required a tracheostomy. None of the 11 patients who developed scoliosis later in life required surgery, but 3 have required bracing. Six of the 11 (55%) required a patch repair for CDH compared to 158 of 264 (60%) CDH patients without scoliosis (p=0.73). CONCLUSIONS: Early diagnosis of scoliosis in CDH patients is associated with a high rate of surgery. There was not a higher incidence of patch repair among patients who developed scoliosis. LEVEL OF EVIDENCE: Prognosis. Retrospective study, level II.