We present a rare case of pleomorphic adenoma in the upper lip region, with erosion of the maxillary bone and distortion of the facial appearance. A 20-year-old man presented with a painless mass on his upper lip, which had gradually increased in size over a period of 2 years. Computed tomography demonstrated a 30 mm × 28 mm enhancing mass in the upper lip region with no invasion to the surrounding tissues. Erosion of the maxillary bone posterior to this lesion was noted. The lesion was excised completely with a wedge of mucosa overlying the tumoral mass, accompanied with abdominal fat grafting to prevent labial asymmetry. Histological examination confirmed the diagnosis of a minor salivary gland pleomorphic adenoma in the upper lip. The pathology, clinical manifestations, and treatment of intraoral pleomorphic adenomas are reviewed.