A case of alveolar soft-part sarcoma located in the uterine corpus is reported. It was an incidental finding in the hysterectomy specimen of a 40-year-old woman. Light and electron microscopic examination revealed periodic-acid-Schiff-positive, diastase-resistant, membrane-bound cytoplasmic granules and crystalloids. Tumour cells expressed immunoreactivity with vimentin, desmin, cytokeratins, NK1/C3 and HMB-45 antibodies. Four years postoperatively, the patient is still alive without evidence of disease. Differential diagnoses, immunocytochemistry and clinical management of uterine alveolar soft-part sarcoma are discussed and the literature reviewed