Low-grade endometrial stromal sarcomas are steroid receptor positive tumors with slow tumor progression and high recurrence rates, which lack established treatment protocols. We present an update on hormonal therapy options.In the past, hormonal therapy consisted of progestins for advanced/recurrent/metastatic low-grade endometrial stromal sarcomas. Aromatase inhibitors and gonadotropin-releasing hormone analogues have become new effective alternatives for first and second line treatment. The high recurrence rates after short disease free intervals in low-grade endometrial stromal sarcoma patients were partly due to inadvertent growth stimulation during estrogen-containing hormone replacement therapy and tamoxifen treatment, which - according to current knowledge - are contraindicated. Recently, hormonal therapy has been introduced for the prevention of recurrences. Aromatase inhibitors are becoming the treatment of choice, since progestins are poorly tolerated due to side effects. The effective duration of preventive hormonal therapy is still undetermined.Hormonal therapy with progestins, aromatase inhibitors and gonadotropin-releasing hormone analogues has become an effective treatment alternative to radiation and chemotherapy for low-grade endometrial stromal sarcoma patients. Preventive hormonal therapy is of particular interest in the setting of concomitant endometriosis.