Anorectal melanoma is a rare cause of anorectal malignancies affecting mainly elderly people without significant gender differences, although there seems to be a white predominance. Diagnosis is often challenging, since symptoms are frequently nonspecific. Radical surgery is the mainstay of treatment, while adjuvant therapies are generally of limited value. Thus, prognosis is still grim, with a 5-year survival rate of less than 20%. We report the case of a 75-year-old white female presenting with mild anal pain and blood in stools. Diagnosed with an ulcerated melanoma of the perianal area, she eventually underwent an abdominoperineal resection and bilateral inguinal lymphadenectomy. To date, she is currently alive and disease-free. Given the lack of adequate international guidelines, we recommend defining a tailored treatment by thorough multidisciplinary discussion, as well as taking into account the patient personal preference.
