Extramammary Paget’s disease (EMPD) was firstly described by H. Crocker in 1889 in a patient with skin lesions of the scrotum and penis. It was later established that it occurs predominantly in areas of the skin rich in apocrine sweat glands. According to the classification, it is divided into primary and secondary, and the latter is associated with adenocarcinomas of visceral organs. The disease’s clinical picture is non-specific and requires differential diagnosis with inflammatory diseases of the genitals. Histological and immunohistochemical examinations are determinative methods of diagnosis establishment. The detection of Paget’s cells identified both by routine microscopy and by using histochemical stains is the histological marker of EMPD is. Immunohistochemical markers are used for differential diagnosis of primary and secondary EMPD: GCDFP-15 (+), CK7 (+) and CK20 (–) are for primary, GCDFP-15 (–), CK20 (+), CK7 (±) can be for secondary depending on the nature of tumor that caused its occurrence. The experience of disease treatment is limited due to the rare incidence. Surgical excision is the treatment method of choice for local lesions without metastases. Recently, experience of the use of topical cytotoxic agents (bleomycin, 5-fluorouracil) and imiquimod in the treatment of non-invasive EMPD has been gained. Systemic chemotherapy (vincristine, docetaxel, carboplatin, 5-fluorouracil, mitomycin C, etoposide) is recommended for metastatic EMPD. Invasion depth and presence/absence of metastases are the main factors determining prognosis.