Primary osseous leiomyosarcoma of the spine is a very unusual condition, with only few cases being reported in the literature. In fact, this type of tumors arises from the smooth muscle cells and occurs usually in the uterus and the gastrointestinal tracts. If the spine should be involved, it occurs generally as a metastatic location. Location to the spine as a primary site is exceedingly rare. We present the case of a 37 years old female patient, with multiple spine levels involvement - to vertebral body and to posterior aspects of Vertebra, causing spinal cord compression syndrome. A secondary location to the skull was diagnosed one month later. Through a literature review, we analyze various aspects in the diagnosis and management of this rare entity.