The central nervous system is traditionally considered an uncommon site for metastatic disease from female genital tract tumors. We report the case of a 48-year-old woman with malignant mixed müllerian tumor of the uterus, who developed spinal cord compression by epidural metastasis a few days after the diagnosis of the uterine malignancy. Emergency decompressive laminectomy was performed and a good recovery of the neurological function was achieved. In the following days, while submitted to extensive staging for the uterine malignancy, the patient complained of headache, confusion and visual disturbance. CT scan revealed multiple brain metastases. No other site of metastatic disease could be detected. The patient refused any further treatment and died 1 month later from progressive cerebral disease. Attention should be paid to the possibility of unusual distant metastases associated to uterine sarcoma in order to treat these patients promptly.