Patient: Female, 74Final Diagnosis: Hiatal hernia with gastric volvulusSymptoms: DyspenaMedication: —Clinical Procedure: —Specialty: SurgeryObjective:Rare co-existance of disease or pathologyBackground:Upside-down stomach (UDS) is the rarest type of hiatal hernia (HH), with organoaxial gastric volvulus. A large HH sometimes causes cardiopulmonary impairments owing to multiple factors.Case Report:We herein report a case of a large HH with UDS that had induced weight loss and severe cardiopulmonary dysfunction in a 74-year-old female patient who presented with shortness of breath, chest pain, severe anorexia, and weight loss of 5 kg over the 3 previous months. Chest X-ray and CT examination revealed that her heart was retracted on the right side, and the hernia contents had induced physical compression of the left lung on the cranial side. Spirometry revealed that the patient’s vital capacity (VC), percentage VC, and percentage forced expiratory volume (% FEV) at 1 s were 1.32 L, 60.2%, and 67.5%, respectively. A barium swallow test confirmed a diagnosis of HH with UDS. On the basis of these findings, we performed a laparoscopic Nissen procedure, which resulted in the patient’s dramatic recovery. Postoperative examinations showed that the stomach and heart were once again normally located, and the left lung had re-inflated. Postoperative spirometry dramatically improved.Conclusions:A large HH causes cardiac and pulmonary compression due to mass effects and leads to cardiopulmonary dysfunction. For cases that have both a complicated HH and cardiopulmonary dysfunction owing to the mass effects of hernia contents, laparoscopic HH repair can be a good alternative procedure.