Adenoid cystic carcinoma is a relatively rare epithelial tumor of the major and minor salivary glands that makes up less than 1% of head and neck neoplasms. The typical clinical and pathological findings of this neoplasm include slow growth, perineural invasion, multiple local recurrences, and distant metastasis. Herein, we report a patient with adenoid cystic carcinoma located to the lower lip which is quite uncommon.