Uterine tumour resembling ovarian sex cord tumour (UTROSCT) are a histological variant of endometrial stromal sarcomas (ESS). There is no established medical management of metastatic UTROSCT or ESS, although there is evidence supporting the use of hormonal therapy. Given the success of aromatase inhibitors in breast cancer, their potential role in ESS and UTROSCT is of current interest. We report the first case of response to second-line, single agent anastrazole in a patient with metastatic UTROSCT.