Orbital and ocular adnexal lymphoproliferative disorders account for 10-15% of orbital mass lesions. This spectrum includes the lymphocytic lesions, plasma cell tumors, histiocytic disorders and leukemic deposits. They represent a continuum of disorders ranging from benign to overtly malignant with considerable risk of systemic involvement. They develop as primary or secondary tumor manifestations in the orbit, conjunctiva, lid, lacrimal gland and sac. Advancing immunohistochemistry and molecular biology allow more accurate immunophenotyping of individual clones of lymphoid cells and early and precise detection of genetic alterations. Specific immunobiologic diagnosis allows ophthalmologists to consider the lymphoproliferative lesions as a distinct clinicopathologic entity from idiopathic orbital inflammatory disease, with much different behavior. Tissue biopsy remains mandatory for diagnosis with thorough systemic medical evaluation by hemato-oncologist. Radiotherapy is of choice for the majority of localized lesions while disseminated disease requires systemic chemotherapy or combined therapy. Recent immunotherapy is promising satisfactory results with low toxicity and risk of relapse. Long-term follow-up with half-yearly examination is recommended for detection of new lesions, late recurrence or systemic affection.