Lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is among the most common malignant lymphomas of the ocular adnexa and it furthermore shows an increasing incidence. The disease is most often localized and indolent of nature; gentle treatment is therefore of essence to avoid complications, especially visual impairment. MALT lymphoma can be treated with radiotherapy (RT), but in case of relapse, alternatives are required. New treatment modalities such as anti-cluster of differentiation (CD)-20 monoclonal antibodies (rituximab) and local injection of interferon-alpha have shown promising results.