Primary central nervous system (CNS) angiosarcoma is a rare form of angiosarcoma, which itself represents 2% of all sarcomas.  Somatic mutations involving angiogenic signaling pathways and other oncogenic pathways in angiosarcoma have been identified, but the mutational landscape of primary CNS angiosarcoma is less well studied.  Recently, recurrent CIC gene rearrangements were found in 9 cases of angiosarcoma (8 primary and 1 secondary), but none initiated in the CNS.  We present here a case of primary CNS angiosarcoma with a CIC-LEUTX gene fusion. The patient is a 43-year-old woman with a history of acute lymphoblastic leukemia, initially treated with chemotherapy, and then methotrexate and whole brain irradiation after CNS relapse.  She eventually underwent T cell-depleted bone marrow transplant with chemotherapy and total body irradiation after developing isolated bone marrow relapse.  Twenty-six years later after a car accident, brain imaging demonstrated 3 incidental meningiomas, 2 of which were resected, with pathology consistent with WHO Grade I meningioma.  Surveillance imaging also demonstrated multiple asymptomatic supra- and infra-tentorial vascular lesions thought to be radiation-induced cavernomas.  One lesion, with homogeneous enhancement located in the left tentorial region with extension to the left midbrain and thalamus, continued to grow on surveillance imaging scans and was resected. Pathology was consistent with primary CNS angiosarcoma with epithelioid features and CIC-LEUTX gene arrangement.  Since surgical resection, the patient has been recommended to undergo adjuvant chemotherapy with temozolomide.  To our knowledge, this is the first case of primary CNS angiosarcoma with this CIC-LEUTX gene fusion, which has only been demonstrated once before in the literature involving a case of a 26-year-old woman with primary angiosarcoma of the thigh. This case adds to the genetic diversity of primary CNS sarcomatous lesions and may benefit from targeted treatment approaches shown to be effective in other CIC-rearranged malignancies.