We report a case of an anomalous origin of the right pulmonary artery (RPA) from the ascending aorta diagnosed at echocardiography at 13 days of age. The diagnostic clue was relieved in the suprasternal and parasternal high short-axis views, showing aorto-RPA continuity with a systolic flow in the left pulmonary artery and a systo-diastolic flow in the RPA. At 34 days of age the infant was submitted to surgery during which a direct end-to-lateral anastomosis without conduit interposition was performed. During the short-term follow-up the patient developed RPA stenosis at the anastomosis site and underwent percutaneous stent implantation.