38-year-old female presented with sudden neurological deterioration 6 years after an operation and chemotherapy for uterine leiomyosarcoma. An extremely rare metastasis of the uterine leiomyosarcoma to the brain was identified and totally resected. Whole brain irradiation (50 Gy) was given. A recurrence of the metastasis was resected 10 weeks later. She ultimately died of a second recurrence. Aggressive surgical management of cerebral metastasis of uterine leiomyosarcoma may achieve an improved outcome.