Fibroblast growth factor (FGF) and its receptor (FGFR) signaling pathway is a key molecular hub that regulates cell proliferation, differentiation, migration and survival, playing a core role in various physiological processes such as embryonic development, tissue repair and metabolic homeostasis. Abnormal activation of this pathway, especially driven by genetic abnormalities such as mutations, amplifications or chromosomal rearrangements of FGFR family genes (FGFR1-4), has become an important oncogenic factor in the occurrence and development of many malignant tumors. Patients carrying such variations have a poor overall survival prognosis. In recent years, with the deepening of the concept of precision medicine and the popularization of high-throughput sequencing technology, the development of new drugs targeting EGFR variations has made continuous progress. Several small molecule inhibitors have been approved for the treatment of tumor patients with specific FGFR gene abnormalities, significantly improving the prognosis of these patients. This review aims to explore the correlation between the FGFR signaling pathway and tumor occurrence and development in tumor patients and the treatment options for tumor patients with FGFR signaling pathway variations, with a focus on the new progress in FGFR-targeted therapy.