Adenoid cystic carcinoma is a common malignant neoplasm originated from salivary glands. The clinical and pathological findings typical of this tumor include slow growth, perineural invasion, and potential local recurrence. It accounts for nearly 2% to 4% of head and neck area tumors. In minor salivary glands it usually affects the palate. We described herein the case of a patient who presented a posterior tongue lesion, which was treated with surgery and radiotherapy as additional treatment. We also carried out a brief literature review on the subject.