Liposarcoma is the most common soft tissue sarcoma inadult life while esophageal liposarcoma is an extremelyrare tumor.In the world literature,only 14 cases ofesophageal liposarcomas have been described.We reporta 72-year old male patient who was urgently admittedto our hospital for acute epigastric pain with a burningretrosternal sensation,persistent nausea,vomiting anddysphagia.Barium swallow,upper gastrointestinal(GI)endoscopy,esophageal manometry and CT scan,failedto accurately diagnose the lesion.After surgical resectionof an esophageal polypoid tumor,the histologicalexamination revealed a well-differentiated grade Iliposarcoma.Diagnostic and therapeutic tools were dis-cussed and the results of literature were reviewed. Liposarcoma is the most common soft tissue sarcoma inadult life while esophageal liposarcoma is an extremelyrare tumor. In the world literature, only 14 cases of esophageal liposarcomas have been described. We reporta 72-year old male patient who was urgently admitted to our hospital for acute epigastric pain with a burning retrosternal sensation, persistent nausea, vomiting and dysphagia.Barium swallow, upper gastrointestinal (GI) endoscopy, esophageal manometry and CT scan, failed to accurately diagnose the lesion. After surgical resection of an esophageal polypoid tumor, the histologicalexamination revealed a well-differentiated grade Iliposarcoma . Diagnostic and therapeutic tools were dis-cussed and the results of literature were reviewed.