Mutations in the neurofibromin gene (Nf2), encoding merlin, are associated with the autosomal dominant multiple syndrome called neurofibromatosis type 2.4 Merlin is an upstream activator of Hippo,5 and currently, Nf2 is considered the only gene of the Hippo pathway that is found to be inactivated via a mutation in cancer, as for example, in vestibular schwannoma. Here, NF2 is linked to NF2-related schwannomatosis.