Over the past 11 years (1974 to 1985) ten patients with perianal Paget's disease were treated. The average age was 64 years and half were male. Two patients were diagnosed as an incidental finding after hemorrhoidectomy and the remainder presented with a symptomatic perianal rash (itching and moisture) that averaged two years in duration. Physical examination in these patients demonstrated characteristic lesions (seven with erythematous or ulcerated, whitish gray lesions and one with a papillary lesion). Three patients presented with invasive carcinomas and, despite aggressive therapy, all developed metastatic disease. Two patients had local excisions with minimal margins and developed associated invasive cancers at four and ten years after diagnosis. The remaining five patients were treated by wide local excision and skin grafts. At present all are free of disease. The characteristic appearance of this lesion and its failure to respond quickly to conventional therapy should lead the clinician to obtain a biopsy which readily establishes the diagnosis. Experience confirms that wide local excision is adequate therapy, but adequate initial evaluation and close follow-up are necessary to identify other malignancies that may develop.