Background: Primary broad ligament leiomyosarcoma (LMS) is an extremely rare tumor with few well-documented cases. We are aware of 15 previously reported observations.
Aim: Report of a new case.
Case: In this report we report a case of a right pelvic sidewall leiomyosarcoma with involvement of the appendix in a 49-year-old woman. A hysterectomy, bilateral salpingo-oophorectomy, omentectomy and appendicectomy were done, without complications. The patient died five months later. The case illustrates the difficulty of correct diagnosis before intervention. On this occasion, review of the literature confirms that this site remains unusual and the management is difficult.