Five cases of esophageal granular cell tumor are reported with characteristics similar to those of benign tumors described previously. In contrast to previous reports these tumors are suitable for removal by endoscopic means, provided they are small and are strictly submucosal in location. This view is further supported by the still open discussion as to histogenesis, and the contradictory opinions on its biological significance. Owing to the increasingly widespread use of fiberoptic instruments the diagnosis “granular cell tumor” will become more common. The diagnosis must always be confirmed histologically. Since the tumor may invade adjacent tissue, or even show a tendency to malignant degeneration in late stages, endoscopic removal seems necessary.