We report a rare case of concomitant presentation of partial anomalous pulmonary venous connection and type A interrupted aortic arch in a 4-week-old, 2.1 kg newborn. She presented in extremis and was found to have a closed ductus arteriosus with the entire left pulmonary venous return obstructed at its connection with the innominate vein. Emergent operative repair was performed in one-stage, consisting of aortic arch reconstruction and anomalous vein translocation. Postoperative recovery was unremarkable and the patient was discharged from hospital 12 days after operation.