Unilateral pulmonary artery discontinuity (UPAD) is a rare malformation which is associated with other intracardiac abnormalities. Cases accompanied by other cardiac abnormalities are often missed on prenatal echocardiography. We reported our case which was prenatally diagnosed as pulmonary atresia with ventricular septal defect (PAVSD) and left pulmonary artery (LPA) discontinuity.