Autoimmune diseases are a group of diseases that can result in organ-specific and/or systemic (systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis,...) anatomical deformation and loss of function as a result of the body's immune system attacking its own antigens under the influence of genetic and environmental factors. The eye is a potential target for complications related to autoimmunity, and depending on the severity of the immune response, vision-threatening problems can be observed. Immune complexes accumulate in and around the vessel walls as a result of humoral immunity, in which the autoantibody-related complement system is activated, and cellular immune response can lead to retinal vasculitis, vaso-occlusion, retinal hemorrhage, retinal ischemia, ischemic optic neuropathy, and inflammation of periorbital tissues. Therefore, the role of ophthalmologists in disease management is important in diagnosing, investigating, and treating ocular symptoms in a timely manner. Early diagnosis and treatment can play a critical role to decrease the risk of irreversible immunological damage to the retinal tissue. A multi-disciplinary approach for complete management and evaluation is needed in this disease group. This study aimed to provide a detailed review of the current clinical perspective for the diagnosis, workup, and treatment of autoimmune diseases with retinal manifestations. Systemic lupus erythematosus, Polyarteritis Nodosa, Dermatomyositis, Scleroderma, Wegener's Granulomatosis, Churg-Strauss Syndrome, Takayasu Arteritis, Susac Syndrome, and Nodular Panniculitis are discussed under this heading.