Ectopic sebaceous glands in the esophagus are a very rare anomaly which have, until now, been described in only 14 living individuals. We describe here the macroscopic, pathological, and clinical features of three new endoscopically diagnosed cases of this benign condition. One of the three patients showed a very high number of lesions (more than 100) distributed throughout the esophagus. A review of the existing literature is presented and the possible pathogenetic mechanisms involved are discussed.