Adenoid cystic carcinoma is one of the most common malignant tumors in salivary gland. Its clinical features contain progress slowly, typically with perineural extension and local invasion. Lymphatic metastasis is rare and blood ciruclation metastasis is relatively common. Surgical excision followed by radiation is the most common course of treatment for ACC, however, chemotherapy is usually used for the advanced patients and the patients that progress fast or not suitable for surgery or radiation. There is no standard chemotherapy regimen. Although with good prospect, target therapy and immunotherapy are still in its infancy, and more clinical trials are wanted.