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vmj.sagepub.com Unilateral absence of the pulmonary artery is a rare con-genital anomaly that is encountered at a rate of 1/200,000.1,2 Pulmonary artery agenesis can occur on either side, fre-quently on the right side, and usually co-exists with cardiac anomalies such as tetralogy of Fallot and septal defects.3 This condition is frequently diagnosed in the neonatal period or in early childhood in patients suffering from shortness of breath, coughing, and frequent pulmonary infections. A 21-year-old male patient without significant medical history had been suffering from shortness of breath and mild thoracic pain for 2 months. The patient was evaluated by conventional radiography and computed tomography (CT) angiography of the chest. Postero-anterior chest radi