There are several subsets of GISTs with features distinct from those of conventional KIT- or PDGFRA-mutated GISTs, including pediatric GIST, neurofibromatosis type 1 associated GIST (NF1-GIST), Carney–Stratakis syndrome, the Carney triad, and familial GISTs (Table 1):Pediatric GISTs, which are predominantly found in the female stomach, are frequently associated with predominant epithelioid features, lymph node metastasis, and mutations in the SDH complex. The gene discussed is SDHB; the disease is neurofibromatosis type 1.