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A 50-year-old woman was admitted to our hospital due to multiple lung nodulesdetected incidentally on a chest X-ray. A video-assisted thoracoscopic lung biopsyrevealed low-grade endometrial stromal sarcoma (LG-ESS). She had undergone asimple hysterectomy 1 year earlier owing to a diagnosis of adenomyosis. A review ofher previous hysterectomy specimen showed not endometriosis but LG-ESS. Accordingto the patient’s levels of serum follicle stimulating hormone and estradiol, shewas in the premenopausal state with retained and normally functioning ovaries. Shethen underwent ovarian ablation by radiotherapy, after which she was administered2.5 mg of letrozole once per day. Three months later, the size of the metastaticnodules in both lungs had decreased. The patient was followed up for 24 monthswhile continuing on letrozole, and maintained a partial remission. We report hereinon a case of metastatic LG-ESS treated with letrozole after ovarian ablation by radiotherapy.