Although several mouse models for NF1-associated neurofibromas and NF2-associated schwannomas have been developed and successfully used to explore tumor biology and evaluate promising therapies (Chen et al., 2019; Gehlhausen et al., 2015; Giovannini et al., 2000; Mayes et al., 2011; Wu et al., 2008; Zheng et al., 2008; Zhu et al., 2002), PSC engineering offers the opportunity to generate humanized mouse models of these tumors. This evidence concerns the gene NF2 and schwannoma.