A case of malignant lymphoepithelial lesion of the submandibular gland is reported and the literature is reviewed. This neoplasm has been described frequently in Eskimos and is usually located in the parotid gland. The histogenesis of the lesion is discussed. The tumor shows local aggressiveness, with frequent recurrences and metastases to regional lymph nodes. Surgical treatment consisting of wide resection of the tumor with regional lymph node dissection, with or without radiotherapy, seems to be the most appropriate therapy.