The lower lip is a very rare emerging site for pleomorphic adenoma. The present review is to accentuate the definition, frequency, clinical and histopathological features, differential diagnosis, prognosis, and treatment choices of pleomorphic adenoma. We reviewed the English-language literature addressing pleomorphic adenoma of the lower lip. The tumors of minor salivary glands are not frequent among whole salivary glands tumors. Pleomorphic adenoma is the most common neoplasm among the minor salivary glands. A wide excision is recommended if there is no cosmetic care and no risk of damage to the functional structures of head and neck. Malignancy of the minor salivary glands are not rare. In addition, malignant transformation of pleomorphic adenoma involved in the benign tumors of minor salivary glands are possible. Thus, awareness of the possibility of existence of a pleomorphic adenoma or a malignant tumor within a mass located on the lower lip has a mentionable importance.