In a 7-day-old infant referred because of bile-stained vomiting, jaundice and lack of meconium, radiological examination revealed the 'double-bubble' sign of duodenal atresia as well as dextrocardia. This infant also had a strawberry haemangioma on the right shoulder. Operation disclosed situs inversus and a preduodenal portal vein as well as duodenal atresia. A side-to-side duodeno-jejunostomy was performed successfully without damage to the anomalous vein. The history of polyhydramnion during gestation, the presence of other anomalies, the rapid onset of bile-stained vomiting and the classic 'double-bubble' sign, together appeared to indicate that the duodenal atresia was intrinsic and not due to the external pressure of the anomalous vein on the duodenum.