Pulmonary atresia with ventricular septal defect is a complex congenital heart disease. We report a case of a 46-year-old woman with pulmonary atresia and ventricular septal defect, surgically treated when she was 15 years old with an implant of a conduit between ascending aorta and left pulmonary branch. A 64-slice computed tomography was performed to assess the conduit patency. The contrast enhanced MDCT study showed the conduit was pervious and correctly connected with an enlarged left pulmonary artery implant of a conduit between ascending aorta and left pulmonary branch.