Brain metastases of uterine leiomyosarcoma are very rare. In this report, we present a rare case of uterine leiomyosarcoma with brain metastasis who presented with diplopia, headache and right facial paresis. Cranial magnetic resonance imaging (MRI) revealed mass lesions that occupy the right side of the pons and the left inferior part of the frontal lobe. Whole-body F-18-FDG PET-CT scan showed hypermetabolic activity in the uterine mass, lungs, peritoneal cavity, right gluteus muscle and in the right posteriolateral portion of the pons. Tru-cut biopsy was performed from the uterine mass. Histopathological examination of the biopsy specimen revealed pleomorphic leiomyosarcoma. Initial presentation of uterine leiomyosarcoma with neurological symptoms is very rare.