Paraneoplastic retinopathy and paraneoplastic optic neuropathy comprise a heterogeneous group of ocular syndromes associated with various clinical symptoms and multiple circulating antiretinal antibodies. Current evidence supports an underlying autoimmune mediated process, which is the rationale for the provision of immunosuppressive therapy in addition to antitumor treatment. There are no controlled clinical trials that address the treatment of paraneoplastic retinopathy and/or optic neuropathy. Management decisions must be based on a relatively small number of case reports. There have been no reports of spontaneous visual improvement in these disorders. Therefore, any improvement after treatment is attributable to the therapeutic intervention. With the exception of the paraneoplastic optic neuropathy patient group, most patients show little or no response to immunosuppressive therapy, and only a small percentage of patients have dramatic improvement. However, modest improvements in visual function can improve patient quality of life and functional independence. Prompt diagnosis and initiation of therapy before significant visual loss is seen seems to be a critical factor in treatment success. An increase in serial autoantibody titers may serve as a marker of disease activity and allow initiation of therapeutic interventions before symptomatic visual decline.