Fibroblast growth factors (FGFs)/Fibroblast growth factor receptors (FGFRs) signaling has recently been found to play very important roles in bone development and diseases. Missense mutations in several FGFs and FGFRs were found in human to cause a variety of congenital bone diseases including chondrodysplasia syndromes, craniosynostosis syndromes, and syndromes with dysregulated phosphate metabolism, etc. In addition to its role in bone development and genetic diseases, FGF signaling is also involved in the maintenance of adult bone homeostasis and fracture healing, etc. Understanding the underlying molecular mechanisms for the role of FGFs/FGFRs in the regulation of bone development and diseases will ultimately lead to better prevention and treatment of FGF signaling-related bone diseases. In this review, we are going to give a summary of the role of FGFs/FGFRs in skeleton development and diseases, and pay more attention to those recent progresses.