Granular cell tumor is a benign tumor, commonly found in the skin, tongue, and breast but rarely in the esophagus. A 44-year-old man was referred from the private clinic because of an esophageal lesion found on endoscopy. Esophagoscopy revealed a white-yellowish polypoid lesion covered with normal looking mucosa in the upper esophagus. It was movable within the wall by pushing with forceps. Endoscopic polypectomy after ligation with elastic "O" band was performed without complication. Histologic diagnosis of granular cell tumor was made. It was stained strongly positive for S-100 protein. Another 20 cases of esophageal granular cell tumors reported in Korean literature were reviewed. (Korean J Gastrointest Endosc 2003; 26:8489)