The loss of the SMARCB1 protein expression, in addition to occurring in EpS, is also a hallmark of other neoplasms, notably of some rare paediatric tumours, including malignant rhabdoid tumour (MRT) and atypical teratoid and rhabdoid tumour (ATRT), both of which have a poor prognosis and arise in children with a mean age of diagnosis of 1–2 years [4, 5]. The gene discussed is SMARCB1; the disease is rhabdoid tumor.