Granular cell tumor (GCT) of the esophagus is a rarely-met-with neoplasm. It is almost invariably benign in character and is of still-disputed histogenesis. Small lesions are asymptomatic. Those over 1 cm in diameter may be accompanied by painful swallowing or retrosternal discomfort. Local resection is the treatment of choice for lesions that are accompanied by symptoms, or that are larger than 1 cm. A tumor incidentally encountered in an asymptomatic patient may be safely followed endoscopically. A successful endoscopic removal of such a lesion is described.