Survival is significantly compromised in infants born with congenital diaphragmatic hernia and major cardiac anomalies. Mortality is highest when congenital diaphragmatic hernia occurs in association with d-transposition of the great arteries. We present three infants with congenital diaphragmatic hernia associated with d-transposition of the great arteries from a single institution. All three infants survived to discharge after surgical repair/palliation of both the diaphragmatic hernia and heart defect and are doing well at last follow-up. The clinical course and management of these three patients are described.