Benign metastasizing leiomyoma (BML) is a rare hormone-dependent disease which occurs predominantly in women during their child-bearing years. After our patient had refused ablative hormone therapy (bilateral ovarectomy), evidence of estrogen and progesteron receptors in tumor tissue taken from the lung sites, as well as extremely high estradiol serum levels, led us to conduct high-dosage antiestrogen therapy for 5 years; daily administration of 250 mg of Tamoxpuren(R) resulted in stable disease of the pulmonary sites without any side effects. This also significantly lowered estradiol serum levels, which improved clinical symptoms. Five years later, the patient's vision suddenly deteriorated due to bilateral macula degeneration. This forced us to stop the antiestrogen therapy and commence alternative treatment with LHRH analogue (3.6 mg Goreselin). We observed stable disease of the pulmonary metastases and low estradiol serum levels during the first 6 months of Goserelin treatment. The response to antiestrogen therapy in BML suggests that the muscular component of these disorders is responsive to estrogen ablation.