The frequency of NF2 mutations increased significantly with the WHO grade: 81/220 of grade 1 (36.8%), 265/441 of grade 2 (60.1%, p = 0.0001) and 122/176 of grade 3 (69.3%, p = 0.0001) meningiomas harbored NF2 mutations. The gene discussed is NF2; the disease is meningioma.