Deleterious germline mutations in LRBA gene encoding lipopolysaccharide-responsive, beige-like anchor protein (LRBA) have been recently associated with an autosomal recessive monogenic disorder, whose common denominators are LRBA deficiency, Autoimmunity, regulatory T (Treg) cell defects, Autoimmune Infiltration, and Enteropathy (LATAIE syndrome) [1–5]. This evidence concerns the gene LRBA and Abnormal intestine morphology.