Extramammary Paget disease is a rare disease. Different treatment options are used for its management, among them treatment with imiquimod. Our aim is to clarify the efficacy and safety of the use of topical imiquimod cream in the treatment of extramammary Paget disease. In this article the current literature in Pubmed and Scopus on this topic is reviewed. According to the literature the topical use of imiquimod can be used as first-line treatment in different dosages and durations with excellent results, reaching a 87.5% cure rate with rare complications. The available evidence is currently limited to case reports and a single case series, but is definitely in favor of imiquimod use as an alternative treatment for extramammary Paget disease.