We presented a 25-year-old patient with symptoms and signs of acute abdomen who has been followed up with the diagnosis of neurofibromatosis type 1 since 1993. Findings of sonography and abdomino-pelvic computed tomography showed a heteroechogenic pelvic mass that arises from the uterus extending to the umbilical level. The patient underwent laparotomy for pelvic mass. On intraoperative observation, the mass which was originated from the retroperitoneal region of pelvis was infiltrating the posterior portion of the uterus and extending to the parametrium. The subtotal hysterectomy and bilateral salpingoophorectomy was performed The histopathological diagnosis of the tumor was malignant peripheral nerve sheath tumor.
Keywords: malignant peripheral nerve sheath tumor, neurofibromatosis type 1, uterus.