Adenoid cystic carcinoma (ACC) of the breast is a rare neoplasm accounting for 0.1% of all breast carcinomas, and presenting most commonly as a painful breast mass. In contrast to the aggressive nature of ACC at other sites, ACC of the breast has a favorable prognosis, lymph node involvement or distant metastases seldom occur. Treatment is basically of simple mastectomy. Chemotherapy, radiation and hormonal treatment have been infrequently used and evaluated. We report a case of ACC of the breast managed with mastectomy and review the literature.