This case report describes a rare and unusual lesion found in a 12-year-old boy, which was diagnosed as pleomorphic adenoma of the minor salivary glands in the upper lip. The tumor was a circumscribed, submucosal nodule, about 2.0 cm in diameter and characterized by slow growth and a rubbery consistency. Complete excision was performed and the histopathological analysis showed an epithelial salivary gland tumor with islands of plasmacytoid cells, duct-like structures, in a variable stroma with chondroid, fibrous and myxoid appearance. The tumor did not recur. A brief review of the relevant literature is also presented. (J. Oral Sci. 50, 225-228, 2008)