Autoimmune eye diseases are a kind of inflammatory eye disease caused by autoimmune reaction, mainly including autoimmune uveitis, thyroid-associated ophthalmopathy, Sjögren's syndrome-related ophthalmoxerosis, neuromyelitis optica spectrum disorder and so on. The pathogenesis of these diseases is complex, involving the ocular surface, intraocular and orbital lesions. Traditional treatment methods mainly include corticosteroids and immune inhibitors, which have low efficiency and many side effects. Therefore, more in-depth research on the pathogenesis of autoimmune eye diseases is needed in order to find therapeutic targets for the etiological treatment. Programmed death 1 (PD-1) has been confirmed to be associated with many autoimmune diseases, and PD-1/programmed death ligand 1 (PD-L1) pathway plays a key role in the regulation of immune responses. Inactivation of the PD-1/PD-L1 pathway may lead to abnormal activation of autoimmune T cells, leading to the occurrence and development of autoimmune diseases. In this review, the immune regulation mechanism of PD-1/PD-L1 pathway that has been discovered at the present stage, as well as its role in autoimmune ophthalmopathy, is systematically summarized, and the potential of targeted treatment of autoimmune eye diseases in the future is prospected. [ABSTRACT FROM AUTHOR]