Sarcomas are tumors that arise from mesenchymal tissue. The nervous system has multiple tissues of mesenchymal origin and thus may serve as an origin for sarcomas. In the brain, these tumors are most often in continuity with the dura. Primary intracranial leiomyosarcomas are the rare tumors that originated from the mesenchymal cells of the dura mater or cerebral blood vessels. There is few report of secondary metastasis to the scalp, calvarium and dura mater. Although leiomyosarcoma accounts for only 2% to 4% of childhood soft tissue sarcomas, the prognosis is poor in HIV-infected compared with non-infected patients. The development of Epstein-Barr virus (EBV)-associated SMT in children with acquired immunodeficiency virus (AIDS) decreases health, reduces quality of life, and often results in death.