In the general population, 1 in 500 people will develop a sporadic schwannoma (1 in 1,000 will develop a vestibular schwannoma and 1 in 1,000 will develop a nonvestibular schwannoma).9 While the majority of heritable predisposition to schwannomas is known to be associated with NF2 germline variants, a proportion of these have also been associated with LZTR1 variants.1,2 In our review of 1,556 people with proven 22q11.2DS (89% with LZTR1 deletion) none had a schwannoma. Here, LZTR1 is linked to schwannoma.