CIC-rearranged sarcomas represent a distinct pathologic entity of undifferentiated small round cell sarcomas of bone and soft tissue. CIC::NUTM1 fusion is pathognomonic for a small subgroup of CIC-rearranged sarcomas, which was first described in 2016, and since then further 13 cases have been clinicopathologically reported. This subgroup often occurs in axial location with frequent bone involvement, while central nervous system is affected only rarely with three cases reported in the literature. Herein, we report a case that expands this limited cohort of patients with CIC::NUTM1 sarcoma of the brain.