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]. The gene discussed is SMARCB1; the disease is brain cancer.