NKX proteins are the most important type of NKL homeobox transcription factor subclass and have been found to be widely involved in regulating cell fate and body pattern formation during embryonic development, especially embryonic development of nervous system, and involved in the occurrence of thyroid cancer, lung cancer, prostate cancer and other tumors. In view of the important roles of NKX proteins in embryonic development and tumorigenesis, the research progress of the structural properties, physiological mechanisms, and disease correlation of 17 proteins in the 7 NKX homeobox protein families were systemati-cally summarized, which would provide helpful information on gene targeted therapy of related diseases. In addition, the lack of genomics research and animal model construction of diseases related to some NKX genes were also discussed, which should be directions for future research.