This idea is in line with the fact that homozygous loss‐of‐function variants in the LUBAC components HOIL‐1 and HOIP were identified to result in a syndrome encompassing autoinflammation and immunodeficiency with variable manifestation depending on the site of the mutation (Boisson et al, 2012, 2015; Nilsson et al, 2013; Wang et al, 2013; Oda et al, 2019). Here, RBCK1 is linked to Immunodeficiency.