The eye is an important receptor for the information we obtain, and common diseases include refractive error, cataracts, retinal disease, and vitreous opacities. But there is also a rare but not uncommon disease of the eye, lymphoma. Ocular lymphoma often occurs in the appendages of the eye, such as the lacrimal gland, conjunctiva, orbit, eyelid, etc. It can also occur in the retina. The most common type of lymphoma is mucosa-associated lymphoid tissue extra-nodal marginal zone B-cell lymphoma, which is often referred to as MALT lymphoma, while other types of lymphoma are less common. MALT lymphoma is an inert lymphoma that progresses slowly and can remain asymptomatic for many years if it occurs in other areas, but the eye is a very sensitive area and many patients often have uncomfortable symptoms that are detected early. Common symptoms include localized swelling in the eye and pressure symptoms caused by the swelling, such as conjunctival swelling, eyelid swelling, diplopia, protrusion of the eye, loss of vision, and limitation of eye movement. If an MRI or CT examination is performed, localized occupying lesions can be found, often compressing the surrounding tissues. Patients with ocular lymphoma often have symptoms first and then visit the ophthalmology department, where MRI and other tests are performed to find the mass. Because lymphoma is a systemic malignant tumor of the hematologic system, a hematologist must conduct a comprehensive examination and evaluation after the diagnosis is confirmed and formulate a treatment and follow-up plan, including various blood tests, whole-body PET/CT, bone marrow examination, evaluation of vital organs before chemotherapy, clinical stage of lymphoma, etc. The treatment plan includes combination chemotherapy, immunochemotherapy, etc. If the optic nerve is involved, cerebrospinal fluid examination or intrathecal chemotherapy is required. In cases with optic nerve involvement, cerebrospinal fluid examination or intrathecal chemotherapy is required, while those with complete resection and no other lesions can be followed up. The prognosis is mostly good, but it varies depending on the extent of the disease, stage, risk factors, etc. Patients who have completed treatment should also visit the hospital for regular follow-up and observation.