Abstract A case of alveolar soft part sarcoma of the uterus is described. It presented as intermenstrual bleeding in a 47-year-old woman. This is a rare tumor, forming about 1% of soft tissue sarcomas. Several hundred cases have been described, mostly arising in the limbs. We believe this to be the 3rd case reported in the uterus and the 12th in the female genital tract. The other reported cases are reviewed, and further information about their outcome has been obtained from the authors.