The treatment strategy for extramammary Paget's disease depends on the presence of metastasis. For the group of patients with primary tumor limited to the epidermis, the aim of treatment is local control by precise detection of the tumor margin. For patients without indications for surgery, radiotherapy or photodynamic therapy would be the treatment of choice. On the other hand, the prognosis of patients with distant metastases is desperate. Although a limited response to combination chemotherapy was observed, no survival benefit has been proven yet. New modalities of treatment such as molecular targeting drugs and hormonal therapy are expected.