Four patients with NF1-mutant high-grade glioma arising in the context of neurofibromatosis type 1 (NF1 c.7258 + 1G > A and p.N420fs; NF1 p.A1523fs*30 and NF1 p.D2163fs*16; NF1 c.1527 + 4_1,527 + 7del; NF1 c.1063-2A > G and NF1 c.5205 + 1G > A) and two patients with sporadic NF1-mutant high-grade glioma received trametinib monotherapy (NF1 p.R1294*, NF1 p.R1947*). The gene discussed is NF1; the disease is neurofibromatosis.