New data have recently been reported in ocular adnexal lymphomas (OALs), particularly of mucosa-associated lymphoid tissue (MALT)-type, regarding their biological characteristics and therapeutic management. A possible association between OAL and Chlamydia psittaci infection has suggested new mechanisms of lymphomagenesis and opened the way for specific targeted treatment. Similarly, the place of rituximab monoclonal antibody therapy and more conventional chemotherapy, as well as a 'wait-and-see' policy in few clearly defined situations, must be defined in relation to the standard treatment option consisting of radiotherapy for low-grade lymphomas. The aims of this review are therefore to present the various treatment modalities and to discuss the place of each modality in the management of ophthalmologic lymphomas.