Abstract
Primary intracranial leiomyosarcomas are rare tumors that arise from the mesenchymal cells of the dura mater or cerebral blood vessels Here we report the case of an extra axial leiomyosarcoma in the right parieto-occipital region of a 19-year-old male who had normal clinical and laboratory findings. Diagnostic imaging showed bony destruction, dural involvement and no parenchymal invasion. No primary site was found after metastasis work up. Specific serology tests were negative. The patient underwent a craniotomy, total tumor resection, duraplasty and skull reconstruction. He received radiotherapy and after 18 months of follow-up, no clinical and radiological signs of recurrence have been found.