Purpose: The incidence of the primary intracranial leiomyosarcoma is extremely rare, and few cases have been previously reported worldwide to date. This report was to clarify the potential role of radiotherapy in the management of primary intracranial leiomyosarcoma. Methods and Materials: This report presented a 49-year old man with a 3-month history of a progressively headache and walking unsteadily. The diagnosis was confirmed with thalamic leiomyosarcoma of high-grade malignancy according to the pathologic examination after neurosurgical biopsy. The patient didn't undergo surgical resection because of a high risk death. After biopsy, radiotherapy using 3D-CRT technique to the mass site with 55.8Gy/31f/43d was given accordingly. Results: The mass didn't reduce much at the end of radiotherapy. The patient refused systemic chemotherapy, he was alive without signs of local relapse and brain side-effects with 6 month's follow-up. After living eleven months and three weeks after radiotherapy, he died of local progression. Conclusions: Through literature review, the current therapeutic approaches including surgery, radiotherapy as well as chemotherapy appear to have limited effect, but could be beneficious of patients in tumor local control and improvement of the life quality. © 2014 Lifescience Global.