Autoimmune retinopathy is a rare autoimmune disease that primarily affects retinal photoreceptor function. It mainly presents in the fifth and sixth decades. Three main forms of autoimmune retinopathy (AIR) have been identified: cancer-associated retinopathy (CAR), melanoma-associated retinopathy (MAR), and non-neoplastic autoimmune retinopathy (npAIR). In this chapter, the term AIR will be used to encompass all three disorders. Patients typically present with a sudden onset of photopsia, rapid visual loss, and abnormal electroretinograms (ERGs). Different types of AIR present with similar clinical features and it requires extensive work up to rule out other differential diagnosis. Pt presents with poor visual prognosis that may be due to delayed diagnosis and delay in initiation of treatment. Different treatment modalities have been tried, including systemic immunosuppression with steroid and steroid-sparing agents, intravenous immunoglobulin, and plasmapheresis, with variable results. Different types of antiretinal antibodies have been found in these patients with autoimmune retinopathy such as antibodies to recoverin, __ampersandsignalpha;-enolase and transducin-__ampersandsignalpha;, but seronegative disease is also common. A lot of research work has been done in this field to understand the pathophysiological mechanisms that is responsible for autoimmune retinopathy, but than also understanding about this rare disorder is limited. In this review we have tried to summarize the pathogenic mechanism, clinical features, investigation, differential diagnosis, treatment and prognosis of autoimmune retinopathy.