A rare case of the potentially grave combination of lung cancer and partial anomalous pulmonary venous connection (PAPVC) is described. PAPVC would cause many problems following major lung resection, even in a preoperatively asymptomatic patient, because of the inevitable development of right ventricular failure as a result of right ventricular volume overload caused by the left-to-right physiologic shunt. On the other hand, if a patient has primary lung cancer, anatomical resection should be done to achieve curative treatment. We successfully performed a left lower lobectomy for lung cancer in a patient with abnormal venous drainage in the left upper lobe, with simultaneous correction of a PAPVC.