Infiltrating syringomatous adenoma of the nipple is a distinct, benign clinical entity. It is similar histologically to a syringoma, a benign tumor originating in the ducts of the dermal sweat glands. When located in the nipple, this lesion has been mistaken for nipple duct adenoma or tubular carcinoma. Infiltrating syringomatous adenoma of the nipple is locally infiltrating but does not metastasize. Appropriate local management depends on an accurate diagnosis. Following is a case report, review of the literature, and therapeutic options for infiltrating syringomatous adenoma of the nipple.