A case of right diaphragmatic hernia is presented associated with a complex congenital heart disease (double-outlet right ventricle, transposition of the great arteries and left isomerism) diagnosed prenatally. Despite high-frequency oscillatory ventilation plus nitric oxide and uneventful repair of the hernia, the infant died after 6 days of uncontrolled pulmonary hypertension and severe aortic coarctation that developed postnatally.