Extramammary Paget disease (EMPD) is a rare tumor, which typically involve genitalia. The lesions are often similar to chronic dermatoses, which complicates the diagnosis of the disease. Skin biopsy with histological and immunohistochemical examination is required to verify the diagnosis of EMPD. In most cases, surgical resection accompanied by follow-up is used, but the relapse rate is still quite high. The paper focuses on the pathogenesis, diagnosis and treatment of EMPD and reports a case of a female patient with EMPD, who was diagnosed 5 years after the onset of the disease.