This case report describes a rare and unusual lesion found in a 33-year-old male, which was diagnosed as pleomorphic adenoma of the minor salivary glands in the upper lip. The tumor was a circumscribed, large firm mass, about 3 cm in diameter, almost obstructing the nares and characterized by slow growth. Complete excision was performed and the histopathologic analysis showed pleomorphic adenoma. The tumor did not recur. A brief review of the relevant literature is also presented.