Abstract： The nuclear factor I（NFI）family is a group of important transcription factors，consisting of four members：NFIA，NFIB，NFIC，and NFIX. The members of the NFI family are highly conserved in the N-terminal DNA binding and dimerization domain. The NFIX gene is expressed in many parts of the body，including the brain，prostate，muscles，skin，fat，and ovaries.It plays an important role in a variety of biological processes，including nervous system development，tumorigenesis，muscle and bone development，hematopoietic cell proliferation，and spermatogenesis.Currently，there are few studies and clinical reports on the NFIX gene in China，and its specific mechanism remains unclear，requiring further investigation. To draw the attention of clinicians and enhance the understanding of NFIX gene associated diseases，this article summarizes recent literature and reviews the function and clinical progress of the gene.