Abstract： Extramammary Paget′s disease(EMPD)mostly occurs in the elderly with predilection for the anogenital region. Since its early lesions are similar to eczema and dermatitis, it is easy to misdiagnose. The first?choice treatment of EMPD is surgical excision. However, EMPD is difficult to completely remove and recurrence is common because it often has multiple lesions with obscure margins and extends beyond the clinically visible margin. Other effective treatments include radiotherapy, chemotherapy, photodynamic therapy, immunomodulatory therapy, molecular targeted therapy, and so on. The prognosis of EMPD is mainly associated with the depth of tumor invasion, local lymph node metastasis, lymphatic and vascular metastases. Sentinel lymph node biopsy can serve as a guide to lymph node dissection, and selective prophylactic lymphadenectomy is helpful for subsequent treatment with an increase in survival rates.