A 61-year-old patient had a skin lesion on the right nipple for 1 year. The lesion was a well defined, brownish, crusted, and keratotic papule, and was associated with an underlying walnut sized, fixed, hard mass and a palpable ipsilateral axillary lymph node. Histologic findings were consistent with Paget's disease. Results of immunoperoxidase staining technigues for S-100 protein and carcinoembryonic antigen were negative. After a modified radical mastectomy and chemotherapy, the patient has been in remission for 12 months.