Malignant lymphoma of the ocular adnexa has been reported on for many years, but many steps forward have been recently made. This paper highlights the staging strategies and treatment options based on a review of the most updated and relevant bibliography.A relevant improvement in the management of ocular adnexal lymphoma is represented by PET, which improves the diagnosis and the staging of the disease. Acquisitions have been made in the treatment: low-dose radiotherapy is confirmed for primary orbital mucosa-associated lymphoid tissue lymphoma; oral chlorambucil is proposed as an alternative; immunotherapy is proposed for the treatment of systemic disease. Interestingly, the role of Chlamydia psittaci as the possible cause of mucosa-associated lymphoid tissue lymphomas and the efficacy of doxycycline for the treatment of ocular adnexal lymphomas have been investigated with promising results. A large series of natural killer/T-cell lymphoma has been described and its lethality despite aggressive conventional chemotherapy has been confirmed.Orbital lymphoma is the most common malignant tumor of the orbit and its incidence is increasing proportionally with the rise of the average survival rate of the general population. The combined efforts of orbital surgeons, hematologists, oncologists and radiotherapists have lately produced a mass of new information that can effectively improve the management of orbital lymphoma.