Extramammary Paget disease (EMPD) is a rare neoplasm that arises in skin rich in apocrine glands, such as the axillae and anogenital region and usually affects the elderly. In most cases, EMPD is an apocrine carcinoma in situ, but it can be associated with internal malignancy spreading to overlying skin. Surgical excision with margin control is the generally accepted standard of care. A 74-year-old woman presented with a 1 year history of a pruritic eczematous eruption in perineum which on biopsy was diagnosed as EMPD. Because of the location and extent of the tumor, any surgical approach would have been problematic. Imiquimod 5% cream applied three times weekly for 16 weeks induced complete resolution. Topical imiquimod appears to be a promising treatment option for EMPD, especially when surgery is a challenge, but only a few cases have been reported.