The hyperimmunoglobulin E syndrome is a primary immunodificiency disorder characterized by recurrent staphylococcal infections and markedly elevated serum IgE level. Clinical features are coarse face and severe infections of the skin-furunculosis or chronically pruritic dermatitis and sinopulmonary tract infection from infancy by coagulase positive Staphylocaccus aureus, or Candida albicans etc. The patient’s serum IgE level is elevated but the basic immunologic pathogenesis not fully understood. We have experienced a case of hyperimmunoglobulin E syndrome in a 2 6/12-year-old boy who had suffered from recurrent staphylococcal pneumonias and abscesses and chronically pruritic dermatitis from 1 month of age with elevated serum IgE level. A brief review of the related literature is presented.