Background:Sebaceous carcinoma (SC) is an uncommon neoplasm that usually occur in the skin of head and neck region with a predilection of periocular sites. This tumor is thought to arise from sebaceous glands in the skin and thus may arise anywhere on the body where these glands exist. However, the noncutaneous SC in where the sebaceous glands are anatomically absent have also been reported sporadically, and the pre-existing ectopic sebaceous glands are speculated to be the basis of its origination. Though ectopic sebaceous glands are not uncommon in esophagus, primary SC in esophagus has not been reported thus far. Aims: To report for the first time one primary esophageal SC in a 66-year-old man, investigated its clinicopathological features, diagnostic points and differential diagnosis from other malignancy. Methods:Clinical and radiological data were collected; the tumor morphology and immunohistochemical pattern were analyzed. Related literatures were reviewed. Results:The tumor cells diffusely expressed P40, CK5/6, AR and P53, and EMA staining highlighted the sebocytes with a foam-like pattern in cytoplasm. With the auxiliary immunohistochemical staining, we finally made an unequivocal diagnosis of SC. Conclusions:SC is a rare entity in esophagus which have not been reported before and may bring up diagnostic puzzles to pathologists. The tumor morphology is complex and with a similar immunohistochemical expression profiles just like SC reported in other sites. This unique tumor occur in esophagus extends the spectrum of extraocular, extracutaneous SC from existing case reports.