Melanoma–astrocytoma syndrome (MAS) is a rare autosomal dominant tumor–predisposition syndrome linked to germline mutations in the CDKN2A gene, characterized by an increased risk of multiple tumors, including cutaneous melanoma, astrocytoma, nerve sheath tumors, pancreatic cancer, and squamous cell carcinoma of the oropharynx [29]. Here, CDKN2A is linked to nerve sheath neoplasm.