Introduction: In recent years, the prognosis for low-grade lymphoma in the ocular region has improved markedly, whereas high-grade lymphoma is still a challenging disease to manage. Numerous strategies are available for treating ocular adnexal lymphoma, and these include chemotherapy, radiotherapy, and immune-modulating agents.

Areas covered: This review provides an overview of the available treatment modalities for ocular adnexal lymphoma, including novel treatment approaches and recent advances. Furthermore, management guidelines for ocular adnexal lymphoma are provided based on existing guidelines described for lymphoma outside the ocular region. This review was based on an extensive MEDLINE search using keywords such as ‘Ocular adnexal lymphoma AND treatment’, ‘Ocular adnexal lymphoma AND management’.

Expert commentary: Ocular adnexal lymphoma is a rare disease and the treatment modalities of ocular adnexal lymphoma are numerous. Therefore, we suggest that the treatment should be centralized in centers in each country and placed in a small number of experienced hands. This, in combination with large multicenter studies, will provide new information and improve the prognosis in the future. The treatment of low-grade low-stage lymphoma mainly includes radiotherapy alone. High-stage or high-grade lymphoma should be managed with chemotherapy or immunochemotherapy, potentially in combination with radiotherapy. In recent years, many new therapy approaches have occurred, and expectations for therapies such as chimeric antigen receptor-modified T-cells are high.