Extramammary Paget carcinoma (EPC) is considered to originate from the eccrine and apocrine glands of the adnexal skin of the axilla, vulva or penis, scrotum, or perineum. If immunohistochemical techniques with the use of cytoskeletal markers are applied in addition to histologic examination, EPC lesions are found to be multifocal adenocarcinoma of the adnexal sweat glands. So far, therapeutic approaches have included primary surgery, followed optionally by radiation therapy. It is possible to achieve a cure only when manifestations are detected early and confined regionally. The role of palliative chemotherapy has not yet been assessed in this disease, particularly because of the lack of compliance and follow‐up in patients with EPC. Cancer 1992; 70:704–708.