We report a case of a fetus with hypoplastic left heart syndrome in addition to an anomalous vessel extending from the descending thoracic aorta to the basal segments of the lower left lung without sequestration. The concurrence of these 2 congenital abnormalities is extremely rare and unreported in the existing literature. The anomalous vessel to the left lung may cause increased pulmonary vascular resistance, which has implications for the long-term management of hypoplastic left heart syndrome via the Fontan procedure.