IL10RA and inflammatory bowel disease: Mutations in IL-10R inducing IL-10 signaling pathway defect were the first monogenic defects identified in children with infantile-IBD.[7,8] Here we report the first case of infantile inflammatory bowel disease with heterozygous mutations in interleukin 10 receptor subunit alpha (IL-10RA) in Taiwan, highlight the initial presentation of this disease and review the up-to-date information of diagnostic and therapeutic approach for infantile-IBD.