Partial anomalous venous return (PAPVR) and bronchial atresia (BA) represent rare congenital abnormalities of the lung. Missed diagnosis and misdiagnosis are very common in these patients. Although usually distinct entities, it appears that, in rare cases, they may co-exist owing to inter-related complex embryogenic development. We report a case of a 59-year-old male with both PAPVR and BA that were incidentally detected during a CT pulmonary angiogram and review the literature to suggest the pathogenetic developmental mechanism for this entity. This case demonstrates the utility of multidetector dual-energy CT in delineating the vascular and bronchial anatomy of this complex lung and vascular anomaly. Although uncommon, radiologists should be aware of PAPVR and BA and the coexistence of these two rare lung congenital abnormalities.