Autosomal recessive (AR) interleukin-1 receptor (IL-1R)-associated kinase 4 (IRAK4) deficiency, together with myeloid differentiation primary response gene 88 (MyD88) deficiency, is a primary immune deficiency that impairs IL-1R and TLR family signaling [3]. The gene discussed is IL1R1; the disease is Immunodeficiency.