Unilateral absence of pulmonary artery is a rare developmental anomaly. Infrahepatic inferior vena cava interruption is a well-recognized but uncommon developmental anomaly. Presence of both these anomalies in a single individual is extremely rare. A 58-year-old man with a history of recurrent lower extremity deep vein thrombosis and venous insufficiency presented to our emergency department with bilateral calf pain and swelling. Ultrasound demonstrated extensive deep vein thrombosis throughout bilateral lower extremities. Computed tomography angiography showed smooth tapering of the right pulmonary artery with absent distal most segment. To our knowledge, there is only 1 case report in the literature so far with both the abnormalities present in a single individual.