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Ocular adnexal mucosa-associated lymphoid tissue (OA-MALT) lymphoma is one of the B lymphoid malignancies that originates in the orbit and shows indolent progression with a predominantly localized stage. Radiotherapy appears to be recognized as the most common treatment for localized OA-MALT lymphoma. Surgical resection, eradication of Chlamydia psittaci (C. psittaci) based on the reports of the existence of C. psittaci DNA, and ‘watchful waiting’ are also selected in some cases. Systemic immunochemotherapies including anti-CD20 antibodies may be another option for advanced stages of OA-MALT lymphoma. However, due to the lower frequency of this disease and the difficulty of conducting large-scale clinical trials, no randomized, controlled trial evaluating these treatments has been reported. Therefore, the optimal treatment strategy for OA-MALT lymphoma has not been established. This review provides an update on treatments and discusses the appropriate treatment for OA-MALT lymphoma.