Xanthoma is an uncommon nonneoplastic lesion resulting from the accumulation of histiocytes. It predominantly shows cutaneous manifestations associated with dyslipidemia. However, xanthoma of the esophagus is extremely rare. To the best of our knowledge, only 14 cases have been reported thus far. The clinical significance of this lesion has not been established. However, this lesion should be distinguished grossly from ectopic sebaceous glands and small subepithelial tumors such as carcinoid and granular cell tumor. Moreover, signet ring cell carcinoma, which contains round cells with abundant cytoplasm and has similar histologic features to xanthoma, should be distinguished microscopically.