CIC-rearranged sarcomas represent a distinct pathologic entity of undifferentiated small round cell sarcomas of bone and soft tissue. 1 CIC::NUTM1 fusion is pathognomonic for a small subgroup of CICrearranged sarcomas, which was first described in 2016, 2 and since then further 13 cases have been clinicopathologically reported. [3][4][5][6][7][8][9] 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. 5 Herein, we report a case that expands this limited cohort of patients with CIC::NUTM1 sarcoma of the brain.A 6-year-old male without significant medical history was admitted to hospital for headache of the frontal region bilaterally, subfebrile temperature and vomiting. There was a mass (54 × 48 × 34 mm) detected on magnetic resonance imaging (MRI) in a parieto-occipitotemporal region of the brain arising from the trigone of the left lateral ventricle (Figure 1F). Five days later, the surgery was performed, and the entire tumour was resected. Postoperative MRI and PET/CT (positron emission tomography and computed tomography) did not reveal any signs of residual tumour or metastatic spread of the disease.