Inflammation involving the retinal vasculature may occur as an isolated finding unrelated to an identifiable underlying diagnosis, termed primary retinal vasculitis, or may be associated with a myriad of both ocular and systemic pathology. Retinal vasculitis is most accurately defined as vascular leakage and staining of vessel walls on fluorescein angiography, with or without the clinical appearance of fluffy, white perivascular infiltrates in the eye with evidence of inflammatory cells in the vitreous body or aqueous humor. Management of retinal vasculitis requires a dualistic approach: one, to control ocular inflammation and reverse a potentially sight-threatening condition, and two, to spare no effort in identifying and treating concomitant, and in many cases, life- threatening systemic disease.