Retinal vasculitis is inflammation of retinal blood vessels typically resulting from infection or immune-mediated inflammatory processes. It may present as isolated ocular inflammation or as a part of severe or potentially fatal systemic disease. Ocular complications of retinal vasculitis include cystoid macular edema, neovascularization, tractional retinal detachment, and vitreous hemorrhage, which all greatly threaten vision. Multimodal imaging and thorough systemic investigations are the main tools for making a precise diagnosis, which aids in predicting disease prognosis and visual outcome as well as preserving a patient’s vision and possibly their life. This review aims to discuss the current understanding of retinal vasculitis as well as current diagnostic tools and treatments.