Autosomal-dominant hyper-IgE (AD-HIES) is mainly characterized by eczematous dermatitis, staphylococcal skin abscesses, connective tissue defects, and elevated serum IgE. This disorder is largely associated with heterozygous dominant-negative mutations in STAT3 gene. Herein, we reported a patient with AD-HIES suffering from dental abnormality and allergic reactions.