Adenoid cystic carcinoma (ACC) is a rare malignant neoplasm of the breast accounting for approximately 0.1% of all breast carcinomas with a total 200 cases reported in the literature. In contrast to the aggressive nature of ACC in other organs, ACC of the breast is known to have an excellent prognosis. Recently we experienced the first case of ACC of the breast at Yeungnam University hospital. The case was diagnosed by preoperative fine needle aspiration cytology. We report a case of ACC of the breast managed with breast conserving surgery and review the literature.