Pleomorphic adenoma is common benign neoplasm of the major salivary glands. It usually presents as a painless, slow-growing mass. A 55-year-old male patient visited our clinic with a 1-year history of palpable mass on left upper lip. He didn’t complain any pain but soft tissue swelling around the mass was seen. He had no history of trauma. Complete Excision of mass was done. Histopathological findings exhibited well circumscribed tumor surrounded by a fibrous capsule. Tumor contained epithelial and myoepithelial cells with myxoid stroma, consistent with pleomorphic adenoma. Pleomorphic adenoma(PA) is a well-described benign tumor of the salivary glands originating from myoepithelial and intercalated duct cells. It occurs most often between the ages of 30 and 60 years and is found more commonly in females than in males. Head and neck irradiation is one of risk factor. It is most commonly found in major salivary glands, but can be found in minor salivary glands. Palate is the most common site of minor salivary glands followed by lip. Histopathologically, PA is composed of neoplastic myoepithelial cells intermingled with neoplastic ducts and stroma. Treatment is complete excision. Prognosis is usually excellent. However, malignant change of untreated PA of minor salivary glands occurs up to 3%. Therefore, adequate diagnosis and treatment is required. Herein we report a case of pleomorphic adenoma on upper lip with reviewing literature.