Autosomal dominant hyper-IgE syndrome is a rare primary immunodeficiency (PID) with multisystem pathology [1], caused by dominant-negative loss-of-function (LOF) mutations in signal transducer and activator of transcription factor 3 (STAT3) [2, 3] (STAT3-HIES). This evidence concerns the gene STAT3 and inborn error of immunity.