Ocular adnexa are rarely involved in lymphomas. Four cases are reported of lymphoma involving the ocular adnexa. In two cases a previous systemic involvement existed or simultaneous to the ocular involvement; another patient had systemically a different histological pattern to that observed in the ocular region, and the fourth patient only had ocular region, and the fourth patient only had ocular symptoms; we therefore recorded two cases of primary ocular lymphoma. The lymphoma was located at the orbit in two cases and at the conjunctiva in the other two cases; the condition was bilateral in three cases. The most common symptom was exophtalmus; other major symptoms were diplopia and disturbances in ocular motility. The histological examination revealed three low grade lymphomas and one case of intermediate grade. Management of patients included radiotherapy and different chemotherapeutic regimens with a good response in all cases. A higher ocular relapse rate was observed in those patients with systemic involvement; in one primary case the condition resolved spontaneously. A review of the literature on clinical, diagnostic and therapeutic issues is made.