Autoimmune retinopathy (AIR) is a group of immune-mediated retinopathies that usually results in severe loss of vision and visual field defects.AIR mainly includes paraneoplastic and non-paraneoplastic syndromes.One main feature of AIR is the presence of circulating anti-retinal antibodies (ARAs) in peripheral blood, which are produced through anti-tumor responses, anti-microbial responses, and immune responses induced by autoantigen fragments following retinal injury, and mainly attack retinal photoreceptor cells.ARAs are important for the diagnosis, progression assessment and treatment outcome of AIR.These ARAs often appear before the diagnosis of cancer and can be helpful for the early detection of malignant tumors.The mechanism of ARAs production, its pathological role in AIR, and its significance in clinical practice were reviewed in this article.