Opinion statement: Melanoma-associated retinopathy is a rare paraneoplastic disorder that is challenging to diagnose and even more difficult to treat. Because of the rarity of the disease, therapy is based on analysis of case series and case reports. Based on evidence from these reports, first-line therapy is cytoreduction of metastatic disease through metastasectomy, chemotherapy, and radiation. This can be combined with intravenous immunoglobulin. For refractory visual symptoms, additional therapies include systemic corticosteroids and plasmapheresis, but the success of these strategies has been limited. Because of the rarity of the disorder, new therapies should be evaluated and reported in the literature to expand our clinical understanding of this autoimmune disease.