The expression of hormone receptors (HR) is considered a good prognostic marker in uterine sarcoma. Hormonal therapy is widely employed in the therapy of HR positive breast and gynecologic cancers, however, there is little information concerning hormonal therapy in HR positive extrauterine sarcoma. A 55-60 year age group female presented with an estrogen receptor positive metastatic retroperitoneal leiomyosarcoma (LMS). She was treated with four cycles of a combination of Gemcitabine and Paclitaxel. Her disease remained stable for 29 months when tamoxifen was initiated. The patient succumbed to an unrelated malignancy after a total of 44 months of treatment. Despite emerging reports about the potential benefit of hormonal therapy, selective estrogen and progesterone receptor modulators and aromatase inhibitors, for uterine sarcoma, there is a paucity of information regarding the application of these therapies to sarcomas arising at other sites. Our patient survived significantly longer than expected with metastatic retroperitoneal sarcoma. In part this may be due to the survival benefit associated with HR positive tumors, but it may also indicate a role for hormonal therapy which has yet to be explored.