Substantial progress has been achieved in eludicating the molecular mechanisms of malignant transformation, in establishing therapeutic standards and in evaluating innovative treatment strategies in patients with low grade Non-Hodgkin's Lymphoma. Correlation of genetic aberrations and immunologic marker profiles to histopathological entities and the clinical course has lead to a new transatlantic lymphoma classification. Today, application of 6-8 courses of moderately intensive induction polychemotherapy induces partial or complete remissions in 80% of patients in stage III or IV. Longterm maintenance therapy with interferon alpha significantly prolongs progression free survival. Current multicenter trials evaluate the curative potential of high-dose chemotherapy with autologous stem cell transplantation. Recently, several new approaches including new cytostatic drugs, immunotherapy with monoclonal antibodies and gene therapy with antisense oligonucleotides have been developed and have achieved remissions in pretreated patients. In the future, intensification of chemotherapy and the new treatment options may offer the potential for cure in patients with low malignant Non-Hodgkins Lymphoma.