Pleomorphic adenoma is the most frequently encountered benign mixed tumor of the minor salivary gland and usually presents in the parotid; however, in the minor salivary gland, it is more common in the palate. Tumors of the minor salivary glands are uncommon, with the most common intraoral site reported being the hard and soft palate owing to the preponderance of minor salivary glands in this region followed by the lips. Pleomorphic adenoma arising from minor salivary glands of the lips tends to occur at an earlier age than it does at other sites. Pleomorphic adenoma of the lip is a rare neoplasm, and thus its diagnosis requires a high index of suspicion and a long-term follow-up. Here, the authors present a case of pleomorphic adenoma of the upper lip.