An increase in the incidence of anorectal melanoma has recently been noted in the United States. Anorectal melanoma is an uncommon and lethal condition, with a median survival of <20 months. Unfortunately, nonspecific symptoms also attributable to common and benign conditions (eg, hemorrhoids) cause significant delay in its diagnosis. Although it has not been validated by clinical trials, abdominoperineal resection has historically been the treatment of choice for this disease. However, for the past 2 decades, a lack of clear survival benefit has led to a shift toward less-mutilating wide local excisions. Controversy still exists regarding the benefit of radiation therapy and chemotherapy. Furthermore, the value of nodal surgery in anorectal melanoma is unclear. In this article, we review the history and current status of management of anorectal melanoma, with a particular focus on surgical controversies and challenges in optimizing survival.