This article reviews the diagnosis and management of a patient with cancer-associated retinopathy (CAR) secondary to small cell lung cancer who developed a rare presentation of CAR, cystoid macular edema (CME). CAR is a rare cause of unexplained, often rapid, visual acuity and visual field loss. A 67-year-old man with a recent history of photopsias and simultaneous dimming of vision presented to our clinic. Four months before, he had been diagnosed with small cell lung cancer. Retinal examination was normal, but the visual field was severely constricted. He was referred for an electroretinogram and an evaluation by a retina specialist because of a strong suspicion of CAR, which was confirmed. Despite a lack of strong clinical evidence for the treatment of CAR, and no clinical recommendations for CME found in CAR, our patient's CME was initially treated with diclofenac QID and, subsequently, a sub-tenon injection of triamcinolone. Since his diagnosis of CME, he has been chronically managed with diclofenac QID to treat his edema, with relatively stable visual acuity. His visual fields remain highly constricted. CAR is a rare form of painless bilateral vision loss in patients with a history of cancer. Our patient developed CME from his CAR, and was treated with diclofenac and sub-tenon injections of triamcinolone. Despite potential options for treatment, none have demonstrated much efficacy, and the visual prognosis is typically poor.