Background: Extramammary Paget's disease is a cutaneous neoplasm that presents as erythematous crusted patches and plaques reminiscent of contact dermatitis or inverse psoriasis that can be a challenge to treat in a tissue-sparing manner. The most commonly involved site for this rare disorder is the anogenital region. Surgery is considered as the gold standard therapy. In the last years, the topical use of imiquimod cream in the treatment of this condition has been reported.