Introduction: The most common connective tissue neoplasm of the gastrointestinal tract is gastrointestinal stromal tumors (GISTs). Its presentations are usually abdominal pain and gastrointestinal bleeding. Case Presentation: We report a 31-year-old man with abdominal pain presented to our hospital with 10 days history of abdominal pain. After radiologic investigations, total distended stomach paraesophageal hernia and antral deformity were seen in the CT scan. Subsequently, the patient underwent surgery and after releasing the stomach, a large sealed perforated mass was seen in the antrum near the diaphragmatic hiatus and small localized abscess that subtotal gastrectomy and hernia repair was done. Pathologic examination revealed that the perforated mass is a GIST. Conclusions: We report a rare complication of gist that is perforation and the first report of simultaneous paraesophageal hernia and perforated gist.