Extramammary Paget's disease is an uncommon intraepithelial carcinoma of the skin and mucosa usually occurring in the anogenital area. Perianal Paget's disease is a rare entity, often associated with internal malignancies and a poor prognosis. Clinical manifestations of perianal Paget's disease include pruritis (most common), irritation, rash, lump sensation and the lesion shows erythematous, crusted or scaly area which may weep clear fluid. Lichenified, leukokeratotic or leukoplakia-like patches may also develop in some patients. These areas may resemble eczema or contact dermatitis. Many authors recommend surgery as the treatment of choice. Extended surgical excision for non-invasive lesion and excision of rectum or abdominoperineal resection for invasive disease is recommended. The prognosis for non-invasive lesion is excellent, but for invasive lesion is poor.We experienced one case of perianal Paget's disease which recurred after wide excision of vulvar area for Paget's disase, and performed secondary abdominoperineal resection. Now, radiation therapy was done for adjuvant therapeutic modality.