OBJECTIVE: To review dermatofibrosarcoma protuberans (DFSP), which is a soft tissue neoplasm of the skin that can rarely affect the genitals.MATERIAL AND METHOD: We report a case of inguinal DFSP in a 40-year-old male who presented a slow-growing mass adjacent to the spermatic cord.RESULTS: After extensive surgical resection pathological analysis confirmed the diagnosis of DFSP with resection margins affected, so reoperation with margin exeresis and inguinal orchiectomy was required.CONCLUSIONS: DFSP is rarely localized in the inguinoescrotal region and it requires wide excision and sometimes orchiectomy.