We reviewed a series of 13 eyes in 9 cases of intraocular lymphoma with or without intracranial involvement during the past 15 years. The cases were characterized by moderate or no inflammation in the anterior ocular segment and by the presence of vitreous opacity and fundus lesions. Contrary to the accepted view that this disease simulates uveitis with various manifestations, the fundus lesions in the present series showed disseminated or tumorous subretinal lesions suggestive of proliferation of tumor cells. Only a few cases showed retinal vasculitis or retinal exudates. Electrooculograms showed findings suggestive of widespread impairment of the retinal pigment epithelium. Because of poor prognosis and sensitivity to radiation, we advocate early diagnosis and treatment for this disease.