Background: Differential diagnosis for a nodule in the nipple or subareolar area of woman includes both primary neoplasms of breast as well as those from skin and adnexae. Case Presentation: A 32-year-old woman presented with a painless 0.5 cm subareolar nodule of her left nipple that she had noticed for several months, with no associated nipple discharge. A biopsy revealed an infiltrating adnexal neoplasm with features similar to those seen in syringomas commonly occurring in locations such as upper face and pubis. The infiltrating syringomatous adenoma of the nipple occurs almost exclusively in women of all ages and is cured by simple excision. Microscopic appearance of such a rare benign infiltrating neoplasm of eccrine duct origin occurring in woman's breast should not be misinterpreted as more common infiltrating primary breast carcinoma. Conclusion: Infiltrating eccrine syringomatous adenoma should be included in the differential diagnosis of a nipple or subareolar nodule occurring in woman. © 2009 Sarma and Stevens; licensee BioMed Central Ltd.