Paraneoplastic limbic encephalitis (PLE) is a rare disease that is probably caused by an immunological reaction against CNS-structures. It may present with neurological, neuropsychological or psychiatric symptoms. Besides treatment of the underlying neoplastic disease, there is no generally applicable evidence-based treatment. PLE is most frequently associated with certain carcinomas, but its occurrence with Hodgkin lymphoma has also been recognized. Association with non-Hodgkin lymphoma has only been occasionally reported in single cases. We report two additional patients, in whom malignant non-Hodgkin lymphomas of the B- and T-cell lines were detected. Treatment with corticosteroids in one and chemotherapy in the other case were associated with clinical improvement.