Ocular involvement in lymphoma is a relatively rare condition that can result from a primary intraocular lymphoma or an intraocular manifestation of systemic lymphoma. Lymphoma manifestations frequently masquerade as other more benign intraocular conditions including allergic or infectious conjunctivitis, uveitis, multiple evanescent white dot syndrome, acute retinal necrosis, or herpetic retinitis. Accurate diagnosis depends on a high index of suspicion and frequently requires histopathological analysis of specimens, particularly vitreous biopsy, subretinal aspiration, or retinal biopsy with flow cytometry, polymerase chain reaction, or immunohistochemistry methods. Most of ocular lymphomas are of B lineage. Diagnosis is often complex and needs use of paraclinical evaluations. Treatment mainly consists of chemotherapy. It is important to review the ocular manifestations of lymphoma to assist ophthalmologists in prompt diagnosis of ocular lymphoma. And it also helps oncologists to recognize the need for a complete ophthalmic evaluation in the diagnosis, follow-up, and management of patients with lymphoma.