Rhabdomyosarcoma (RMS) is the most common sarcoma in paediatric patients. A perianal site is unusual and is associated with a low cure rate. The few cases of reported perianal RMS have been associated with sequelae. Here, we report the case of a 29-month-old male child who received sequential treatment by surgery, chemotherapy and radiotherapy inspired by Papillon's irradiation of adult anal/low-rectum cancers (including external beam radiotherapy in the gynecological exam position followed by brachytherapy) and who remains in complete remission 49 months post treatment with no sphincter or other anorectal disorders.