A 3 week old neonate developed congestive heart failure after the operation of an omphalocele. The clinical aspect first suggested to us a persistent ductus arteriosus. Cardiac catheterisation then revealed a bronchopulmonary sequestration, a rare congenital malformation. Children usually become symptomatic with chronic respiratory tract infections, although there are also asymptomatic courses. Only a few cases are reported with congestive heart failure being the initial symptom. Many patients show additional malformations--so did our baby with an omphalocele and a mild peripheral pulmonary stenosis. Once the diagnosis is made, resection or lobectomy is the therapy of choice. In the preoperative diagnosis angiography is mandatory to demonstrate the abnormal vascular supply.