Background: Extramammary Paget's disease (EMPD) is a rare skin disease which can be limited to the epidermis and can sometimes also be associated with underlying carcinomas. At clinical examination, lesions are well-defined eczematous areas and have been described typically in the anogenital region. Surgery is the cornerstone treatment. Case Report: In this report the case of a 66-year-old patient presenting with a long-lasting EMPD of perianal region without deep gastrointestinal neoplasia is described. Because of the extension of the lesion, surgery should have led to abdominoperineal amputation, but the patient rejected this option. Three months of daily application of topical imiquimod was prescribed as an alternative treatment. Biopsy-confirmed complete regression could be observed thereafter, and no recurrence has been noted during a 12-month follow-up. Conclusions: This successful treatment of a perianal-located EMPD by topical imiquimod warrants further investigations.

Keywords: Administration, Topical, Skin Neoplasms - drug therapy, Paget Disease, Extramammary - drug therapy, Antineoplastic Agents - therapeutic use, Anal Canal - pathology, Aminoquinolines - therapeutic use, Administration, Topical, Skin Neoplasms - drug therapy, Paget Disease, Extramammary - drug therapy, Antineoplastic Agents - therapeutic use, Anal Canal - pathology, Aminoquinolines - therapeutic use