Abstract: This paper explores the central regulatory role of the Janus kinase-signal transductor and transcriptional activator (JAK-STAT) pathway in a variety of childhood diseases, and looks forward to the potential of JAK inhibitors in the treatment of these diseases. As a key mechanism of cell signaling, JAK-STAT pathway is crucial for cell proliferation, differentiation and functional regulation. When it is abnormally activated, it can induce a series of immune diseases. In the treatment of a variety of adult diseases, JAK inhibitors have shown significant efficacy, providing a new idea for the treatment of childhood diseases. This paper reviewed the relevant literature at home and abroad in recent years, and introduced the research progress of JAK inhibitors in a variety of children immune diseases and hematological diseases, so as to provide reference for the further development and application of JAK inhibitors.