Abstract Fourteen patients with scimitar syndrome, aged from 4 days to 20 years, underwent surgical treatment between September 1980 and August 1988. Patients were separated into two groups. Group A comprised four neonates with heart failure and severe pulmonary hypertension; part of the right lung was supplied by large aberrant systemic subphrenic arteries (ASSAs) in each. Group B included ten patients (nine children and one adult) with mild to moderate symptoms, normal pulmonary artery pressures; only two had ASSAs. In group A, one neonate with multiple ventricular septal defects underwent pulmonary artery banding but later required a lobectomy because of a lung abscess. The other three neonates underwent ligation of ASSAs; two improved rapidly, and one died of sepsis. In group B, all patients survived intracardiac repair and remain asymptomatic during a follow-up of 24 to 108 (mean 54.9) months. In summary, prognosis after intracardiac repair is excellent in patients without pulmonary hypertension. Neonates with heart failure usually improve after ligation of ASSAs, and pulmonary resection is only indicated in patients with intractable pulmonary sequestrations.