Background. Alveolar rhabdomyosarcoma is an uncommon malignant soft tissue tumor rarely found in the genital tract. This tumor is associated with a poor prognosis, especially in the adult population. Equally as rare are non-puerperal uterine inversions secondary to sarcomas. Case. A 21-year-old obese woman was initially evaluated with excessive vaginal bleeding. On exam, a large pedunculated mass protruding from the cervix was seen and biopsy of this mass revealed an alveolar rhabdomyosarcoma. The patient was treated with adjuvant chemotherapy consisting of VAC (Vincristine, Actinomycin, and Cyclophosphamide) for a presumed cervical rhabdomyosarcoma. After five cycles of chemotherapy the patient underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy, at which time a complete uterine inversion was noted with the tumor located at the fundus of the uterus. Final pathology showed alveolar rhabdomyosarcoma of the uterus. The patient then received additional postoperative VAC regimen for a total of 10 treatments and remains in good health with no evidence of disease 20 months from diagnosis. Conclusion. This case report describes the only reported case of uterine inversion secondary to alveolar rhabdomyosarcoma of the uterus and discusses current therapeutic options for adults. © 2004 Elsevier Inc. All rights reserved.