The aortic origin of one pulmonary artery branch, so-called hemitruncus, is a rare congenital anomaly with poor prognosis. In this report, an additional patient is presented. The patient, a 60-day-old male infant with the right pulmonary artery originating from the ascending aorta was operated successfully. Postoperative catheterization demonstrated normal flow to the right lung and pulmonary artery pressure decreased to normal level.