Background: Malignant tumors of the ear are rare. The most common malignant tumors are squamous cell carcinomas and adenocarcinomas. Lymphoma in the ear is rare. Methods: We report 2 cases of a primary presentation of a lymphoma of the ear. The literature since 1947 is reviewed. Results: An 83- year-old woman with an anaplastic large cell lymphoma of the skin of the external auditory meatus and a 75-year-old man with a B-cell non-Hodgkin's lymphoma of the mastoid process are presented. The literature review shows that only 16 cases of lymphomas of the ear have been reported so far. Conclusions: In a case of therapy-resistant otitis and/or peripheral facial paralysis malignancy should be excluded by computer tomography of the mastoid. For histopathological diagnosis unfixed specimens are preferable. Uniform treatment of lymphomas of the ear has not been established. Copyright (C) 2000 S. Karger AG, Basel.