Causative variants for non‐NF2‐related schwannomatosis have been primarily identified in two genes; SMARCB1 (SWI/SNF‐related, matrix‐associated, actin dependent regulator of chromatin, subfamily b, member 1) and LZTR1 (leucine zipper like transcription regulator 1), both located in the chromosome 22q region although these variants only account for 30%−40% of sporadic cases and 70%−80% of familial cases (Evans et al., 2018; Hulsebos et al., 2007; Kehrer‐Sawatzki et al., 2017; Piotrowski et al., 2014). The gene discussed is SMARCB1; the disease is schwannomatosis.