Since Piotrowski et al. (2014) identified LZTR1 variation in schwannomatosis and analyzed the spectrum of pathogenic variants to show that loss‐of‐function (LoF) was the principal disease mechanism, multiple studies have confirmed that LoF variants of LZTR1 are disease‐causing (Bigenzahn et al., 2018; Hutter et al., 2014; Paganini et al., 2015; Smith et al., 2015; Steklov et al., 2018). Here, LZTR1 is linked to schwannomatosis.