The esophageal hiatal hernia is a herniation of an abdominal organ, usually the stomach, through the esophageal hiatus into thoracic cavity. It is a rare disease, usually congenital and frequently associated with gastroesophageal reflux and other congenital malformations in children. It is classified according to their anatomic characteristics as type I (sliding hiatal hernia), type II (paraesophageal hiatal hernia), type III (combined hiatal hernia) and type IV (multiorgan hiatal hernia). We experienced a case of type III congenital esophageal hiatal hernia simulating chest mass on simple chest x-ray because of right intrathoracic stomach secondary to congenital esophageal hiatal hernia and organoaxial rotation in 10 months male. After the operation, he showed an improved general condition and was discharged at the 14th hospital day. We report the case with the brief review of the related literatures.