NF1‐09, considered nonclassic MPNST, had differences in treatment response compared with the classic ones as it was resistant to AURKAi and MEKi treatments, although the latter could be related to the presence of an oncogenic mutation in PIK3CA, and it was sensitive to JQ1, despite being PRC2 WT. Here, PIK3CA is linked to malignant peripheral nerve sheath tumor.