The case of an 89-year-old man who was referred for a painless decrease of vision in his right eye (RE) is reported. Fundus examination of the RE showed an elevated amelanotic lesion located in the posterior pole with an adjacent focal round pigmented lesion. There was also a more peripheral amelanotic lesion extending from 6 to 9 o'clock clockwise inferotemporally. Uveitis workup and imaging studies of brain and orbits were normal. A retinochoroidal biopsy was done and showed the presence of choroidal extranodal marginal zone lymphoma. The patient was treated with external beam radiotherapy. This report presents a review of the literature of all reported cases of choroidal extranodal marginal zone lymphoma.