Serum anti-retinal autoantibodies (ARA) are a group of autoantibodies that bind to retinal auto-antigens with significant biological importance in pathological processes such as retinal degeneration, inflammatory microenvironment formation, and tissue destruction. In recent years, the expression of serum anti-retinal antibodies has been found to be upregulated in patients with various blinding retinal diseases such as age-related macular degeneration, autoimmune retinopathy, and retinitis pigmentosa, closely correlated with the progression of diseases. However, current researches on ARA are incomplete, lacking animal experiments and large randomized controlled clinical trials. As a result, the exact mechanism of ARA is not well understood. Although several studies have demonstrated that serum ARA has an important diagnostic value in hereditary, autoimmune, and degenerative retinal diseases, there still lacks recognized laboratory tests and laboratory indicators with high specificity and sensitivity. Clinical symptoms should be considered when making definitive diagnosis of the diseases. Therefore, clarifying the mechanisms of ARA in retinal dystrophies provides new ideas in early diagnosis and treatments of retinal diseases, which is clinically and scientifically important for the maintenance of visual functions.