Heterozygous germline pathogenic variants in the SMARCB1 and SMARCA4 genes have been reported to cause rhabdoid tumor predisposition syndrome (RTPS), which is characterized by a high risk of malignant rhabdoid formation with the most frequent localizations in the central nervous system or kidneys and is linked to a more unfavorable prognosis compared to those with sporadic rhabdoid tumors [20,21]. The gene discussed is SMARCA4; the disease is familial rhabdoid tumor.