Hyperimmunoglobulin E syndrome(HIES) is a primary immunodeficiency characterized by severe recurrent soft-tissue infections, pneumonias, pruritic dermatitis and markedly elevated serum IgE levels. Pneumatocele and empyema develop as result of recurrent pneumonias. It is inherited in an autosomal dominant fashion, with variable expressivity. We experienced the case of a 10 month old girl with hyper IgE syndrome. Patient was admitted for local ulceration and muscle soreness at the site of BCG inoculation. The diagnosis of hyper IgE syndrome was made because she had a coarse facial appearance, pruritic dermatitis, pneumatocele and markedly elevated serum IgE level with a past history of frequent skin abscess and pneumonia