Paget disease is a rare disorder of the skin of the vulva, comprising less than 1% of vulvar neoplasms. Surgical excision is considered the standard of care. While the invasive variant of Paget disease is rare, frequent local recurrences from non-invasive disease, which are inoperable, constitute a major clinical problem. In this article we report on a 71-year-old patient with recurrent disease treated successfully with imiquimod cream. Symptoms such as local itching subsided shortly after initiation of treatment. Four weeks later, first signs of resolution could be demonstrated colposcopically. Within 8 weeks, complete clinical remission was achieved. Thus, topical imiquimod is not only effective as treatment of vulvar intraepithelial neoplasia, but may also be used for selected patients with recurrent Paget disease as an alternative to repeat surgical interventions.