Alveolar rhabdomyosarcoma is an uncommon malignant soft tissue tumour rarely found in the female genital tract and carries a very poor prognosis especially in adults. A 44 year old premenopausal womanwas evaluated for a lower abdominal mass, intermittent unprovoked vaginal bleeding and weight loss. Examination showed a huge abdomino-pelvic mass, and an irregular cauliflower mass protruding from the cervix, from which a biopsy was taken. Exploratory laparotomy revealed a widespread disseminated uterine tumour with an “omental cake” and involving virtually all the abdominal viscera. Debulkingsurgery was found to be non-beneficial and uterine and omental biopsies were taken. The cervical, uterine and omental biopsies were all diagnosed alveolar rhabdomyosarcoma. Neither chemotherapy norradiotherapy could be started before the client died of fulminant neoplastic dissemination 17days postoperatively.While the optimal management of this rare tumour is unknown, early recognition and diagnosis, and a prompt multimodality treatment approach of surgery, chemotherapy and radiotherapy offers the best chance of cure (Afr J Reprod Health 2009; 13[1]:167-173)