B6-hIL1RAP mice are humanized models generated using gene editing technology. They are constructed by integrating the protein-coding sequence (CDS) encoding the extracellular domain of human IL1RAP protein and the intracellular domain of mouse IL1RAP protein, along with the 3'UTR of the mouse Il1rap gene, into a specific site within the mouse Il1rap gene. This process retains the endogenous gene sequence encoding the mouse signal peptide (aa.1~20). Homozygous B6-hIL1RAP mice are viable and fertile. This model can be used for studying the pathological mechanisms and therapeutic approaches of cancer and inflammatory diseases, and for the development of IL1RAP-targeted drugs.