Syringomatous adenoma of the nipple (SAN) is an extremely rare neoplasm of the breast. We present a case of 41-year-old female with a retroareolar mass in her left breast. The patient has been operated on twice and remains free of disease for 20 months after the second operation. The nosological status of SAN and its relationship to the microcystic adnexal carcinoma of the skin is discussed.