They account for 0.1%-3% of all gastrointestinal neoplasms.3 Gastrointestinal stromal tumors can arise in various organs but most commonly are found in the stomach and small intestine, and often are diagnosed incidentally.4,5 Up to 85% of GIST have gain-of-function variation in KIT or PDGFRa genes.4 The remaining 15% of GISTs are associated with other genetic alterations, including in NF1, SDH, or BRAF genes.6 The gene discussed is NF1; the disease is gastrointestinal stromal tumor.