Autoimmune retinopathy (AIR) is a retinal inflammatory disorder associated with antiretinal antibodies (ARA) that leads to photoreceptor dysfunction, scotomas, visual field defects, and acute or subacute vision loss. The disease is categorized into paraneoplastic AIR and nonparaneoplastic AIR, with the former further divided into cancer-associated retinopathy (CAR) and melanoma-associated retinopathy (MAR). In pathophysiology, retinal proteins have been identified that have antigenic properties, with recovery being specifically localized to the retina, while others, such as α-enolase, are present in retinal and nonretinal tissues. Nonparaneoplastic AIR is the most common form and predominantly affects individuals in their fifth or sixth decades of life, with a higher prevalence in females. AIR usually begins unilaterally but can affect the contralateral eye, leading to bilateral involvement within a few days to 2 months. Common symptoms include subacute visual loss, visual field defects, scotomas, photopsia, nyctalopia, and dyschromatopsia. Fundoscopic findings may show vascular attenuation, diffuse retinal atrophy, pigmentary changes, and optic nerve pallor. The ancillary exams used are binocular indirect ophthalmoscopy, fundus photography, campimetry (VF), electroretinogram (ERG), fluorescein angiography (FA), OCT (optical coherence tomography), and fundus autofluorescence (FAF). The differential diagnosis of autoimmune retinopathy includes hereditary retinal diseases, retinal degenerative disorders, toxic retinopathies, diseases of the spectrum of the white dots syndrome, multifocal choroiditis, and acute zonal occult outer retinopathy (AZOOR). Due to the difficulty of diagnosis, managing this disease is a challenge. Ocular treatment includes local and systemic immunosuppression. A collaborative effort between ophthalmologists and immunologists is vital to advancing our understanding of autoimmune retinopathy and refining its management strategies.