In 1998, Versteege et al. described SMARCB1 biallelic inactivation in rhabdoid tumors [24, 25]. SMARCB1 (also known as BAF47) is a core subunit of the SWI/SNF chromatin remodeling complex which regulates chromatin structure and thus plays an important role in gene expression [26]. Here, SMARCA1 is linked to rhabdoid tumor.