Abstract: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the digestive tract. They originate from interstitial cells of Cajal (ICC) or their homologous stem cells. Most of them are related to mutations in KIT or PDGFRA genes. The incidence, drug resistance rate of targeted therapy, and recurrence rate are increasing year by year, which greatly affect the prognosis of patients. The treatment of GISTs is facing bottlenecks, and searching for new treatment methods has become a current research hotspot of GISTs. ETV1 is a member of the transcription factors of ETS family, which can stimulate the transcription of KIT gene. KIT proteins enhance the expression of ETV1 through MEK-MAPK signaling pathway. The synergistic regulation of ETV1 and KIT positive feedback leads to continuous activation of intracellular signaling pathways in ICC/GISTs, thus promoting the proliferation of tumors. FOXF1 is highly expressed in GISTs, which may be an upstream regulator of KIT and ETV1 and promotes the expression of genes specific to ICC/GISTs. FOXF1 and ETV1 may provide new ideas and directions for the treatment of GISTs. This article reviews the relationship between ETV1, FOXF1 gene expression, and GISTs.