Treatment: The prognosis for extramammary Paget’s disease depends on the stage of the tumor. Disease that is localized to the skin has an excellent prognosis. The treatment of choice is wide local excision. The risk of recurrence is high, and lifelong clinical follow-up is required. The prognosis for disease associated with an underlying adenocarcinoma is dependent on the stage of the underlying tumor. Lesions associated with an underlying malignancy have a worse prognosis. Metastatic disease has a poor prognosis, and various chemotherapeutic regimens have been tried with and without radiotherapy.