About 5% of vestibular schwannomas (which are neurofibromas originating from the SC of the eighth cranial vestibular nerve) are related to inherited Nf2 mutations, whereas the remaining 95% are sporadic.2 Indeed, between 30 and 50% of new cases arise by random genetic mutations.3 However, merlin is mutated at least in 66% of sporadic schwannomas indicating a correlation between this protein and tumor development.4 Here, NF2 is linked to plexiform neurofibroma.