Primary leiomyosarcoma of the fallopian tube is an extremely uncommon neoplasm. We report a 39-year-old premenopausal woman with leiomyosarcoma confined to the right fallopian tube, who is alive without evidence of disease 2 years after surgical resection followed by adjuvant doxorubicin and cisplatin chemotherapy. An accompanying literature review indicates that the clinical signs and symptoms of fallopian tube sarcomas are usually nonspecific and include lower abdominal pain and pelvic pressure. The age at diagnosis varies from 21 to 70 years, with a median of 47 years. Prognosis is poor, however, several longterm survivors have been reported. The primary treatment remains surgical excision, although adjunctive chemotherapy or radiation may be of some benefit.