It is important to recognize supratentorial neuroepithelial tumors with EWSR1-PLAGL1 fusion as a tumor type in which acquired inactivation of SMARCB1 and development of AT/RT features may occur and lead to clinical progression, similar to previously reported examples involving pleomorphic xanthoastrocytoma, ganglioglioma, and ependymoma [3, 5, 9, 11]. Here, SMARCB1 is linked to ependymoma.