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We report a case of genital and perianal Paget's disease which was treated with wide local excision without anorectal dysfunction. A 76-year-old man noticed erythema on his genital and perianal region. Histopathological features of erythema on the genital and perianal region were, consistent with extramammary Paget's disease. A Computed tomography scan revealed a swollen lymph node in the right groin. The patient was treated by a wide local excision with a right inguinal lymph node dissection and a temporary colostomy. We reconstructed the natal cleft by a V-Y island flap. Microscopically, there were no metastases in the right inguinal lymph nodes. We found six cases of perianal Paget's disease without causing disturbance to the anorectal function after surgery reported in Japan. A free split-thickness skin graft frequently develops severe contracture of the graft region which causes the anorectal dysfunction. A flap technique is a good indication of perianal Paget's disease to preserve anorectal function. [Skin Cancer (Japan) 2002; 17: 241-245]

A case of genital and perianal Paget's disease surgically treated without anorectal dysfunction.