Polymorphous low-grade adenocarcinoma (PLGA) is a rare malignant neoplasm arising almost exclusively in the minor salivary glands. PLGA occurs primarily in the oral cavity, especially in the palate, followed by the oral mucosa and upper lip [1,2]. Conditions involving these locations are often presented at dermatological clinics. Therefore, dermatologists should consider this entity in the differential diagnosis of the oral cavity tumors. We present a case of PLGA in the upper lip.