Mammary Paget's disease is an intraepithelial carcinoma present on the nipple and its areola. The tumor typically develops in middle aged women. It is frequently associated or contiguous to an underlying galactophoric adenocarcinoma. The histopathologic and immunopathologic aspects are typical and linked to the presence of Paget's cells. Early diagnosis is required searching for a possible underlying carcinoma. The surgical treatment is directed by the characteristics of the deep mammary carcinoma.