Among germline mutations, the leucine zipper-like transcription regulator (LZTR1), SWI/SNF related, matrix-associated, actin-dependent regulator of chromatin, subfamily B, member 1 (SMARCB1), and protein kinase cAMP-dependent type I regulatory subunit alpha (PRKAR1A) genes have been reported in association with schwannoma development (schwannomatosis) [4]. Here, PRKAR1A is linked to schwannomatosis.