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.

We present a case of a 59-year-old woman with primary extramammary Paget's disease of the vulva, in which a conservative approach to therapy was desired, and who underwent complete and stable remission with imiquimod cream. We also review the previous reports of patients with extramammary Paget's disease treated with imiquimod cream.

Imiquimod therapy may be an alternative for primary as well as recurring extramammary Paget's disease. Treatment-associated morbidity is minimal compared with other therapies, such as surgery which may be debilitating.