A 67-year-old woman had experienced intermittent vomiting. She was admitted to our hospital because the symptom did not improve. An upper gastrointestinal barium study, computed tomography and gastrointestinal endoscopy showed esophageal hiatal hernia through which a large part of the gastric body herniated into the mediastinum. Therefore, we diagnosed upside down stomach with gastric volvulus in a mesenteroaxial form. Attempts at endoscopic repair failed to reduce the stomach, and laparoscopic surgery was performed. The enlarged esophageal hiatus was sutured with a mesh reinforcement, and Toupet fundoplication was performed as an antireflux procedure. The postoperative course was uneventful, and the patient was discharged on the postoperative day 14. Recently we sometimes encounter cases of upside down stomach in the literature, but this disease is rare and only 41 cases have been reported in Japan. We herein report such a case successfully treated by laparoscopic surgery.