The identification of DCAF5 as an E3 ligase that specifically degrades SMARCB1-deficient SWI/SNF subunits also makes a compelling case for targeting the WDR domain of DCAF5 as a potential treatment strategy for aggressive childhood cancers with SMARCB1 loss of function, the second leading cause of childhood cancer death. This evidence concerns the gene SMARCB1 and childhood malignant neoplasm.