A largely symptom-free swelling of the right submandibular lymph nodes developed in a 23-year-old woman with hyper-IgE syndrome who had suffered from recurrent staphylococcal abscesses since childhood. Histological examination of the lymph nodes revealed highly malignant non-Hodgkin lymphoma of centroblastic type. Extensive staging tests did not reveal any further tumour manifestations. Because granulocyte chemotaxis is abnormal in hyper-IgE syndrome and the patient also had an infection of an old lung cyst, treatment was restricted to local radiotherapy of the cervical lymph nodes. Since 12 months there has been no recurrence. There is probably no connection between the hyper-IgE syndrome and the development of a malignant non-Hodgkin lymphoma, judging by the results of immunological and immunoelectrophoretic findings.