Though most of the features of autosomal dominant STAT3 LOF HIES reflect a deficient immune system, the non-immunological features viz. dental abnormalities (retained primary teeth, non-eruption of permanent teeth, double rows of teeth), anomalies in midline facial development (prominent forehead, wide nasal bridge, broad nose, prominent mandible), and skeletal abnormalities (bone fractures, hyperextensible joints, scoliosis, and craniosynostosis) reflects the multisystem nature of the disease (1, 6, 11–14). Here, STAT3 is linked to craniosynostosis.