The study involved a literature search of published schwannomatosis‐associated LZTR1 variants, and classification of those LoF variants based on current ACMG/AMP guidelines (Richards et al., 2015) and ACGS guidelines v4.01 2020 (https://www.acgs.uk.com/quality/best-practice-guidelines/#VariantGuidelines), including updates on the application of cosegregation data (Jarvik & Browning, 2016), ACMG classifier PVS1 (Abou Tayoun et al., 2018) and the scaled points‐based system (Garrett et al., 2021; Tavtigian et al., 2018, 2020). This evidence concerns the gene LZTR1 and schwannomatosis.