So far, patients with schwannomatosis and LZTR1 germline mutations have not been reported to exhibit meningiomas, in contrast to patients with SMARCB1 germline mutations (Hutter et al. 2014; Piotrowski et al. 2015; Paganini et al. 2015b; Smith et al. 2015, 2016). Here, SMARCB1 is linked to meningioma.