Brain metastasis in uterine leiomyosarcoma (ULMS) is very rare. Reported here is a 41-year-old woman with ULMS history who presented with multiple lung metastases, as she had undergone total abdominal hysterectomy, bilateral salpingo-oophorectomy, solitary lung metastasectomy, and 4 cycles of adjuvant chemotherapy. A multi-targeted tyrosine kinase inhibitor pazopanib was administered orally for recently developed multiple lung metastases. She has been followed for nearly 2 years without any treatment. With complaints of headaches, magnetic resonance imaging revealed a metastatic lesion in the vermis. The lesion was inaccessible, thus, neurosurgery could not be performed, and radiosurgery with Cyberknife was instead elected to be performed. She died due to massive hepatic and lung metastases after the brain radiosurgery completion.