Although most of the germline SMARCB1 mutations causing rhabdoid tumours occur de novo, familial cases have also been reported, with several affected members harbouring constitutional SMARCB1 mutations and malignant rhabdoid tumours but never developing schwannomas (Sévenet et al. 1999b; Taylor et al. 2000; Ammerlaan et al. 2008). This evidence concerns the gene SMARCB1 and schwannoma.