Unlike most pediatric brain neoplasms, AT/RTs are in terms of genetic alterations intersimilar and nearly all cases present with deletion and/or mutation of the tumour suppressor gene SMARCB1 (INI1/hSNF5) located on chromosome 22 which results in loss of SMARCB1 protein expression [2–5]. This evidence concerns the gene SMARCB1 and brain neoplasm.