Primary sebaceous carcinoma of the major salivary glands is very rare, arising mainly in the parotid. The submandibular gland localization is exceptional and only two cases have been reported in the literature. We report a third case diagnosed in Beaujon's Hospital in a 80 year old woman. Histologically, the tumor was poorly differentiated and lobules contained clear cells, foam cells or adipocyte-like cells, containing lipids stained with Oil Red O. We report the clinical, histological and prognostic features of this rare entity. We also discuss the differential diagnoses that pathologists should discard.