Ils disease is an idiopathic retinal vasculitis that occurs mostly in young men. It is a form of occlusive perivasculitis that often begins in the anterior pole of the retina and later progresses to the posterior pole. The arteries are not involved. The disease is mostly bilateral and usually without vitreous inflammation.Spitznas and colleagues reviewed the status of 316 patients with Ils disease and found that common lesions included neovascularization (84%), hemorrhagic lesions (58%), vaso-occlusive lesions (39%), and extravascular sheath formation (34%). Ocular lesions may begin with retinal edema and later develop perivenous inflammation, peripheral vascular filling defects, and retinal neovascularization. The disease may occasionally begin in the optic nerve papillae and present with signs of diverticular retinal block. Possible sequelae include recurrent vitreous hemorrhage with secondary vitreous contracture and retinal detachment. In some patients, the ocular damage may resolve on its own. Systemic lesions can often occur and lead to impaired vestibular and auditory function, myelopathy, and stroke. The disease can affect a diverse population and can be caused by different etiologies. There is a strong correlation with a positive purified protein derivative test (tuberculin test). The presence of immune complexes in these patients suggests that this is an immune disease. However, immunosuppressive drugs are not effective in treating this disease. In patients with capillary leakage and neovascularization, treatment with laser coagulation of the entire retina can be effective. Patients with recurrent vitreous hemorrhage can be treated with horizontal vitrectomy (pars plana). Retinal arteritis with aneurysms is extremely rare and is characterized by peripheral vascular nonperfusion and uveitis with saccular and fusiform aneurysms involving larger, smaller arteries. Associated lesions also include neuroretinitis, retinal neovascularization, optic nerve papillary edema, and anterior uveitis. Most of this disease is seen in younger patients. Although some of the underlying characteristics are not well understood, it is possible that this disease represents a rare form of damage in polyarteritis. However, treatment with immunosuppressive agents is ineffective. Other less common disorders that occur with retinal vasculitis include retinopathy associated with tumors and intraocular lymphoma. In these diseases, retinal vasculitis may be present as an existing disease. Finally, attention should be paid to the high incidence of idiopathic retinal vasculitis. Epidemiological studies have shown that the prevalence of idiopathic retinal vasculitis fluctuates between 45% and 62%.