The coexistence of congenital diaphragmatic hernia and esophageal atresia with or without tracheoesophageal fistula is extremely rare; only 36 cases have been reported. We report a case of a preterm male newborn infant with left congenital diaphragmatic hernia, esophageal atresia, and tracheoesophageal fistula and review 27 related cases.