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Pulmonary atresia with ventricular septal defect is a congenital heart malformation in which a lack of continuity between ventricles and the pulmonary artery is accompanied by ventricular septal defect. We report a 3-year-old female patient, who presented with a history of shortness of breath and bluish lips. Computed tomography pulmonary angiography showed an absent main pulmonary artery, right pulmonary artery, left pulmonary artery and showed a defect that connects the right ventricle and left ventricle, which concluded a VSD (ventricular septal defect).