A high level of serum IgE is immunologic characteristic of several autosomal recessive IEIs, namely interleukin 6 (IL-6) receptor deficiency, IL-6 signal transducer deficiency, ZNF431 deficiency, DOCK8 deficiency, PGM3 deficiency, CARD11 deficiency, CARD14 deficiency, and Comel-Netherton syndrome [3, 4]. Here, ZNF431 is linked to hyperinsulinemic hypoglycemia, familial, 4.