We show that the mutational background of primary atypical meningiomas to be comprised mainly of NF2 mutants, which frequently co-occur with either chromosomal instability or recurrent p.(Arg383Gln) or p.Arg386His mutations in SMARCB1 (co-occurrence P=1.2 × 10−7, Fisher’s exact test). The gene discussed is NF2; the disease is meningioma.