The role of CDKN2A dysfunction in the pathology of schwannomatosis has not been established but some clues may emerge from the study of the mechanisms involved in transformation of neurofibromas into malignant peripheral nerve sheath tumors (MPNSTs) resulting from bi‐allelic inactivation of CDKN2A (Chaney et al., 2020; Magallón‐Lorenz et al., 2021; Nielsen et al., 1999). This evidence concerns the gene CDKN2A and plexiform neurofibroma.