So far, three cases of esophageal xanthoma have been reported. We describe here endoscopic, microscopic and immunohistochemical findings of two new cases of this rare condition, and a review of five cases of esophageal xanthoma, including our cases. Esophageal xanthomas endoscopically show yellow granular spots or a slightly elevated lesion, and are similar to an ectopic sebaceous gland. Microscopically, an aggregate of foamy histiocytes (lipid islands) is seen immediately beneath the squamous epithelium, particularly between the rete ridges. Immunohistochemically, xanthoma cells are positive for CD68, which indicate a histiocytic origin. The etiology of esophageal xanthoma remains unclear. We emphasize that esophageal xanthomas may have been erroneously diagnosed as an ectopic sebaceous gland on endoscopic examination.