Skeletal and connective tissue abnormalities are commonly found in patients with autosomal dominant hyper-IgE syndrome (HIES) due to heterozygous mutations in STAT3, including craniosynostosis, varying degrees of scoliosis or retained primary teeth.7–13 Patients with craniosynostosis and dental anomalies (CRSDA; MIM 614188) were found to carry recessive loss-of-function variants of IL11RA. 5,14–18 The clinical disease phenotype is characterized by multi-suture craniosynostosis, maxillary hypoplasia, delayed or ectopic tooth eruption, supernumerary teeth and minor digit abnormalities. Here, STAT3 is linked to craniosynostosis.