Pleomorphic adenoma is the benign tumor of salivary glands, which originates from the myoepithelial cells and intercalated duct cells. This tumor is more common in major salivary glands. This case report describes a rare and unusual lesion in a 55-year-old female, 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 was characterized by slow growth and 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. No recurrence was observed 1 year after the surgery.