Hyperimmunoglobulin-E syndrome is one of the primary immunodeficiency with the manifestations of recurrent infections especially with Staphylococcus aureus, characteristic facies, hyperextensibility of joints, multiple bone fractures, scoliosis, and delayed shedding of the primary teeth. It is a multisystem disease of autosomal dominant inheritance. Recently, a new type of hyper-IgE syndrome with autosomal recessive inheritance was identified. Although Th1/Th2 imbalance has been suspected to be a cause of this diesease, it is not clarified yet.