The authors report a case of Hodgkin lymphoma developing in a 4.5-year-old female child with hyper-IgE syndrome. This is one of the few cases of malignancy reported in this syndrome. The patient had severe atopic dermatitis, asthma, recurrent pneumonia, recurrent skin infections, and growth retardation. Immunologic evaluation revealed a high level of immunoglobulin E (7000 IU/mL) and peripheral eosinophilia. She was found to have normal values for serum IgG, IgM, IgA, WBC chemotaxis, serum complement function and normal sweat chloride test. The development of fatal Hodgkin lymphoma in this patient with hyper-IgE syndrome may suggest an increased risk for developing premature malignancies in hyper-IgE syndrome, although the precise immunologic defect is still unknown.