Partial anomalous pulmonary venous return (PAPVR) is a congenital heart disease with a reported incidence of autopsied case. The location of the anomalous pulmonary venous return is usually the right atrium, superior vena cava (SVC), and sometimes the brachiocephalic vein, inferior vena cava (IVC) or coronary venous sinus. Recently we experienced a rare case of PAPVR showing anomalous right total pulmonary venous return to the azygos vein. Furthermore, downward translocation of the right upper lobe bronchus was evident. This rare case is reported along with a review of the related literature.