Schwannomatosis (OMIM 162091) as the result of a germline SMARCB1 missense mutation in combination with the acquisition of an independent NF2 gene mutation and acquired loss of heterozygosity at the SMARCB1-NF2 locus on chromosome 22 was described by Van den Munckhof et al. (2012) and Gossai et al. (2015). This evidence concerns the gene SMARCB1 and schwannomatosis.