SMARCB1/INI1 germ-line mutations were first described in the malignant rhabdoid tumors (MRT) of infancy and atypical theratoid/rhabdoid tumors of the central nervous system and define a hereditary condition known as “Rhabdoid predisposition syndrome” [3-6]. This evidence concerns the gene SMARCB1 and atypical teratoid rhabdoid tumor.