Especially, as correct histopathological classification of paediatric brain tumours can be challenging (Judkins et al, 2005; Haberler et al, 2006), our present findings suggest that testing for the mutational status of INI1 is warranted to exclude rhabdoid tumour predisposition syndrome (RTPS) in previous (Dimopoulos et al, 2006) and future reports on familial ependymoma. This evidence concerns the gene SMARCB1 and ependymoma.