Originally described by Sir James Paget in 1874, Paget's disease of the nipple/areola describes the presence within the epidermis of malignant adenocarcinoma cells. Patients generally present in the 5th or 6th decade with unilateral and sometimes bilateral chronic scaly, eczematoid or ulcerated lesions of the accompanied by an exudative secretions. Other entities that should be considered and excluded before rendering a diagnosis of Paget's disease are squamous cell carcinoma, melanoma and Toker cell hyperplasia. A battery of immunohistochemical stains including CK7, CEA, S100, Melan-A, HMB-45, Her-2 and mucicarmine may be performed in addition to correlation with mammographic findings and examination of the surrounding breast parenchyma for an associated carcinoma.