We report the case of a male neonate who had a prenatal diagnosis of Fallot's tetralogy. He presented with respiratory distress during the second week of life. Chest x-ray showed an enlarged right ventricle and pulmonary edema. Echocardiography demonstrated characteristic features of Fallot's tetralogy. However, cardiac catheterization disclosed that, in this case of Fallot's tetralogy, the left pulmonary artery had an anomalous origin in the ascending aorta. At 23 days of age, the patient underwent total surgical correction of the defects, during which a direct anastomosis was employed without conduit placement. 10 days later, he was discharged. We present the results of a literature review of the epidemiological, physiopathological, clinical, diagnostic, and surgical characteristics of this rare condition.