The loss‐of‐function variants such as T645M are inherited either as homozygous or compound heterozygous states and will cause predominantly immunodeficiency with some immunodysregulation,16 whereas GOF variants, which stop the inactivation of RIPK1, are highly penetrant and are associated with a purely autoinflammatory phenotype.17 Here, RIPK1 is linked to Immunodeficiency.