Biallelic inactivation of SMARCB1 is found in over 90% of several aggressive pediatric malignancies including malignant rhabdoid tumor of the kidney (MRTK), atypical teratoid rhabdoid tumor (ATRT), and renal medullary carcinomas (RMC)5,6. Here, SMARCB1 is linked to atypical teratoid rhabdoid tumor.