Extramammary Paget disease is an epidermal carcinoma with apocrine differentiation originating in the epidermis o secondary to epidermotropic dissemination from a near or distant neoplasm. It usually shows as an eczematiform lesion with well defined margins in areas rich in apocrine glands, such as the axilla, genital and anal areas. Prognosis is defined by two factors: depth of invasion of primary tumour and whether there is an associated tumour. Its non specific clinical signs and slow evolution of the lesions, often delay the diagnosis, which leads to a poor prognosis. The diagnosis of Paget's disease is histological, sometimes requiring an immunohistochemical study. This can orient towards the primary or secondary nature of the disease, but will still need an extension study.