A case of extramammary Paget's disease of the axilla in an 84-year-old patient is presented. No underlying carcinoma was found and the lesion was treated successfully by wide local excision. Immunohistochemical staining showed nuclear immunoreactivity for c-myc and cytoplasmic staining for CEA, EMA, CAM 5.2, EGRF, c-erbB-2 and pan-cytokeratin in all the Paget cells. No immunoreactivity of the lesion was observed for S-100 protein, pan-ras, H-ras, K-ras, and p53 oncoproteins. Further research is needed to establish whether oncoprotein overexpression plays a role in the pathogenesis of extramammary Paget's disease and can be used as a diagnostic or prognostic marker.