After the description of DAVID syndrome as a rare combination of anterior pituitary deficit, mostly ACTHD, with common variable immunodeficiency, we and others found that it is associated with NFKB2 gene mutations affecting specific C-terminal residues of the NFKB2 protein, known to play important roles in immunity and also expressed in the pituitary (Quentien et al., 2012; Chen et al., 2013; Brue et al., 2014). Here, NFKB2 is linked to Immunodeficiency.