Intrapericardial diaphragmatic hernia is a very rare phenotype of neonatal diaphragmatic hernia which is thought to be caused by the developmental failure of the septum transversum. There have been only 10 cases reported since 1980, and among them, only 2 cases were diagnosed in fetal life. We herein report a new case that was diagnosed in fetal life, and pericardiocentesis was performed at 27 weeks of gestation. This is the first case to undergo a fetal interventional therapy. After birth, the patient successfully underwent closure of the hernia, despite severe pulmonary hypoplasia.