Lymphoid proliferations of the uvea can be divided into two main groups: (a) primary uveal tumors and (b) secondary intraocular manifestations of systemic lymphoma. Although the exact frequency of the latter is not known, they are not unusual, particularly in leukemia where intraocular (mainly choroidal) involvement may occur in as many as 80–90% of patients at some point in the course of the disease. Primary uveal lymphoma is rare and, unlike vitreoretinal lymphoma, is usually indolent in nature. Uveal lymphoma can be further divided according to location, primary choroidal lymphoma, primary ciliary body lymphoma, and primary iridal lymphoma, with the former being the most common.