A four-year-old girl with hyper-IgE syndrome is presented. She had a coarse facial appearance, pruritic dermatitis, recurrent skin abscesses, pulmonary infection, spontaneous bone fractures, and an elevated serum IgE concentration. She has been treated with cimetidine, ascorbic acid and trimethoprim-sulfamethoxazole for the last two years and there has been no evidence of a severe infection.