We experienced a case of congenital paraesophageal hiatal hernia[Type;IV in seventeen day-old female and treated through the right thoracotomy, reduction of the herniated viscera ,stomach and some part of transverse colon and omentum by gentle finger push, and narrowing the esopahgeal hiatus. Paraesophageal hiatal hernia accounts for only 5% per cent of all diaphragmatic defects but is a potentially dangerous lesion due to compressed lung by the herniated viscera. Symptoms are related to this, including exertional dyspnea, vomiting, cough, Tachypnea but noncyanotic, etc. Barium study shows that the stomach has herniated into the right pleural cavity. The speckled appearance in the herniated stomach in the herniated stomach was due to food material. It strongly suggests paraesophageal hiatal hernia. The operation was done. We report the case with the brief review of literatures.