The authors report a long term success of bipulmonary transplantation in a 15 year old girl with cystic fibrosis and respiratory failure complicated by severe right heart failure. The operation did not cause any particular problems. After transplantation, the clinical signs of right ventricular failure and echocardiographic right ventricular dilatation regressed in less than one week. The right ventricular hypertrophy also regressed. Echocardiography shows no abnormality after 5 years' follow-up.