A histopathologically proven case of Paget's disease of the breast in a 47-year-old male induced a review of the 32 cases in the world's literature. Paget's disease usually presents in the 5th and 6th decades of life without any obvious racial predilections. The most frequent presenting signs include ulceration, eczema, nipple discharge, bleeding, and crust formation. At the time of presentation, 50 per cent of the patients have a palpable breast mass, positive lymph nodes, or both. When entertaining the diagnosis of Paget's disease of the breast using morphologic characteristics of the cells, the diagnosis of malignant melanoma and Bowen's disease (intraepithelial squamous cell carcinoma) must be included in the differential diagnosis. Certain differences will be seen between these three entities based on specific cell staining and the uptake of certain tumor markers. There is no evidence that this disease behaves differently in males versus females, but the 5-year survival for males is worse: 20 to 30 per cent in males versus 30 to 50 per cent in females. The treatment of this disease must focus on the pathology of the underlying tumor. Current treatment involves modified radical mastectomy or radical mastectomy for Stage I and II tumors. Adjuvant chemotherapy, radiation, and tamoxifen are also used depending on the nodal and receptor status of the tumor.