BACKGROUND: Extramammary Paget disease (EMPD) is a rare intraepithelial neoplasm of the skin characterized by ill-defined margins and high recurrence rates after wide local excision. Although Mohs micrographic surgery (MMS) has been proposed to decrease the rate of local recurrence, the efficacy of MMS for this condition has not yet been established. OBJECTIVE: We sought to assess the efficacy of MMS for the treatment of EMPD. METHODS: A comprehensive systematic review and individual patient data meta-analysis was performed including all available clinical studies and case reports with 5 or more subjects describing the use of MMS for EMPD. RESULTS: Eight studies were identified and included in the current review: 3 retrospective studies and 5 case series. In all, 81 patients with 90 cases of MMS were included from these 8 studies. The overall recurrence rate for EMPD after MMS was 12.2% correlating with an estimated 5-year tumor-free rate of 83.6% by using Kaplan-Meier curve analysis. The treatment of EMPD with MMS resulted in significantly lower recurrence rates than wide local excision in this meta-analysis of 3 observational studies with comparators (odds ratio 0.20; 95% confidence interval 0.05-0.81). LIMITATIONS: Limitations include lack of controlled trials, small sample sizes in the included studies, and publication bias. CONCLUSION: The current evidence supports the efficacy of MMS in the treatment of EMPD. Further controlled clinical trials are needed.