Uveal lymphoma by definition affects the choroid, iris, and ciliary body. The vast majority of cases primarily involve the choroid. Uveal lymphoma can be further characterized as a primary uveal lymphoma or as a secondary intraocular manifestation of systemic lymphoma. Most cases of primary choroidal lymphoma (more than 80 %) are low-grade, extranodal marginal zone lymphoma (EMZL) of mucosa-associated lymphoid tissue (MALT). The remainders are comprised of subtypes such as follicular, lymphoplasmacytic, small lymphocytic, mantel cell, diffuse large B-cell lymphoma, and others. Treatment depends upon the extent of ocular involvement, laterality, and presence or absence of systemic involvement. Therapies may include radiation, immunotherapy, and chemotherapy. Overall survival, particularly with low-grade, indolent forms such as EMZL, is excellent.