Uterine leiomyosarcoma is a malignant smooth muscle tumour of the uterus. It is rare and accounting for less than 2% of cases in malignant gynaepathology. To date, only a few reported cases of leiomyosarcoma arising from leiomyoma documented in the literature. We shared an uncommon occurrence of leiomyosarcoma arising from leiomyoma. Presented herein is a case of a ‘rare epithelioid subtype’ of leiomyosarcoma arising from a leiomyoma in a postmenopausal woman. We highlighted the importance of recognizing the possibilities of this event to allow for a timely diagnosis of leiomyosarcoma and to provide insights on management of patients presented with clinically presumed fibroid.