Adenoid cystic carcinoma (ACC) of the breast is a rare type of primary breast cancer with a favorable prognosis, despite its triple-negative status. There has been no consensus regarding treatments. We will report a case of Adenoid cystic carcinoma of the breast presented with localized disease and no axillary lymph node metastases. We performed a radical mastectomy with axillary lymph node dissection, with a favorable clinical course and we will describe the clinic pathologic features of ACC of the breast and review the current literature.