To date, only one case discusses the diagnosis of a pediatric spinal meningioma in the context of a new neurofibromatosis type 2 (NF2) diagnosis with multiple CNS lesions whereby the dominant intracranial tumor was surgically resected and histopathologically confirmed as a meningothelial grade 3 meningioma with chromosome 22 loss and without TERT mutation and CDKN2A/B deletions; however, this genetic profile was never separately confirmed for the intraspinal tumor [66]. This evidence concerns the gene CDKN2A and NF2-related schwannomatosis.