Congenital heart disease is the most common birth defect, affecting 1%–1.2% of live born infants. Pulmonary atresia with intact ventricular septum (PA-IVS) accounts for <1% of all total heart defects. The cause of PA-IVS has been unclear. Thus, experience for prenatal diagnosis of PA-IVS is limited in any single institution. This is the case of a 28-year-old gravida 1 para 0 who came in at 34 + 5 weeks of gestational age. Fetal two-dimensional (2D) echocardiography revealed Type II PA-IVS, higher risk for univentricular circulation postnatally. She gave birth at term by vaginal delivery, with confirmed findings through a 2D echocardiography. Prenatal diagnosis of PA-IVS allows options for the termination of pregnancy, fetal cardiac interventional therapy, early postnatal initiation of prostaglandin E1, and planned early neonatal interventional surgeries for palliation and repair. Early assessment of fetal cardiac features is useful for a better outcome.