A 64-year-old man presented with a four or five-year history of erythema on the genital area. On the first consultation, erythema and depigmentation, partially with induration, were observed on the genital area. Histological findings of the erythema showed Paget's cells in the epidermis and dermis. A computed tomography scan revealed multiple lymph node swellings in the left inguinal, para-aorta and left subclavicle areas. Based on these findings, a diagnosis of extramammary Paget's disease, stage N was made. The patient was treated with palliative resection of the genitals and left inguinal lesions, followed by low dose FP (a combination of 5-FU and CDDP) therapy. After 3 courses of this therapy, partial response (a 60% reduction in the para-aorta lymph node swelling) was obtained in the lymph node metastases without any side effects. Low dose FP therapy might be one of the effective therapies for advanced extramammary Paget's disease. [
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