The BML is a rare disease with 100 reported cases in the literature. Although it is a rare condition, it should be considered in women with multiple pulmonary nodules. There is no gold standard treatment at the moment due to the limited number of cases. Treatment methods include surgical resection of metastatic lesions, hysterectomy, bilateral oophorectomy and medical castration using hormonal therapy if the tumour is positive for estrogen and progesteronen receptors. Further studies wi l l hopefully discover markers to identify uterine smooth muscle tumours with potention to become benign metastasizing leiomyomas.