LOF mutations in the gene encoding CASP8, a protease that initiates apoptosis and regulates immune responses have been described very recently to cause infant‐onset IBD.155 Previously, patients with CASP8 mutations have been shown to present with autoimmune lymphoproliferative syndrome‐like (ALPS) like disorder.156 In contrast, the novel report shows patients with previously undocumented mutations in CASP8 presenting with severe VEO‐IBD as the main clinical manifestation. This evidence concerns the gene CASP8 and inflammatory bowel disease.