Central retinal vein occlusion (CRVO) is usually seen in older adults and is often associated with systemic vascular disease. CRVO can be seen in young adults, and although it is occasionally associated with a systemic disease, in the majority of cases it occurs in an otherwise healthy patient with no known systemic disease or ocular problem. Inflammation of the central retinal vein has been proposed as a cause of the occlusion in young adults and for that reason it has been called papillophlebitis. The appearance of unilateral optic disc edema, dilatation, and tortuosity of the major retinal veins with a variable amount of retinal hemorrhage in young, healthy adults with complaints of blurred vision or photopsias has been called, in addition to papillophlebitis, benign retinal vasculitis, optic disc vasculitis, nonischemic CRVO, big blind spot syndrome, and presumed phlebitis of the optic disc. An approach to the diagnostic evaluation of the young adult with CRVO is presented. Although most eyes recover vision to better than 20/40, about one-fifth have significant visual loss, and many suffer ocular sequelae. Many treatment modalities have been tried for this entity, but no conclusive evidence exists that any treatment alters its natural history.