Anomalous origin of one pulmonary artery from the ascending aorta is a rare congenital malfomation. To date, there have been 60 reported cases in the literature. Usually the right pulmonary artery arises form the ascending aorta and other malformations such as patent ductus arteriosus are commonly associated. Early diagnosis is essential because congestive heart failure develops in early life and operation is contraindicated if severe pulmonary vascular changes have occurred.