This paper reports development of non-Hodgkin’s lymphoma in a 7'/2-year-old patient with hyper IgE syndrome. This rare primary immunodeficiency syndrome is characterized by markedly elevated serum IgE levels, chronic atypical dermatitis and serious recurrent infections. Laboratory features include exceptionally high levels of IgE, near normal levels of IgG, IgA, IgM, with pronounced eosinophilia
