Ocular manifestations of systemic malignancy are important for both the ophthalmologist and the internist to recognize because they may precede the diagnosis of cancer. This review of the current literature discusses the clinical manifestations, etiology, and potential therapeutic interventions for a group of visual paraneoplastic syndromes, including carcinoma-associated retinopathy and melanoma-associated retinopathy. These conditions are characterized by elevated serum levels of autoantibodies directed against tumor antigen that cross-react with retinal proteins, resulting in rod and cone dysfunction. The clinical presentation, site of origin, frequency, and intraocular distribution of tumors metastatic to the eye are also reviewed.