Acinic cell carcinoma (ACC) is a low-grade malignant salivary neoplasm that constitutes approximately 17% of primary salivary gland malignancies. In the head and neck region, the parotid gland is the predominant site of origin and women are usually more frequently diagnosed than men. Previous radiation exposure and familial predisposition are some of the risk factors for ACC. A slowly enlarging mass lesion in the tail of the parotid gland is the most frequent presentation. The diagnosis is usually confirmed with a fine needle aspiration biopsy, and surgical excision is the main treatment of this malignant neoplasm. Other treatment modalities such as radiotherapy may be indicated in some cases. ACC has a significant tendency to recur, to produce metastases (cervical lymph nodes and lungs), and may have an aggressive evolution. Therefore, long-term follow-up is mandatory after treatment.