Unilateral pulmonary artery agenesis (UPAA) is an uncommon congenital anomaly and most patients present in neonatal period with respiratory symptoms. Left-sided pulmonary artery agenesis is less frequent than right-sided and is sometimes associated with cardiac anomalies. We report a patient with a history of repaired ventricular septal defect, who presented with cough and hemoptysis and the diagnosis of UPAA was made.