Coats disease is an idiopathic retinal vascular disorder with retinal telangiectasia with intraretinal and/or subretinal exudation without appreciable retinal or vitreal traction. The condition is sporadic with no associated systemic abnormalities. The incidence of Coats disease is 0.09 per 100,000 population. Ninety six percent of cases are unilateral, with predominance for young males (80%). No genetic basis has been found, but there has been described chromosomal instability in chromosomes 3 and 13. Clinical features are vision loss, strabismus, xanthocoria, and nystagmus or pain. Anterior segment is usually not involved in early cases. Intraretinal exudation is present in almost all cases and can be widespread and away from the telangiectasia. The exudation tends to gravitate toward the macula. The main causes of poor vision in Coats relate to presence of subfoveal fluid or exudation, subfoveal fibrosis or hemorrhage, macular edema, development of epiretinal membrane, or optic atrophy. In addition to binocular indirect ophthalmoscopy (BIO), which ultimately establishes the diagnosis, OCT, ultrasound, and fluorescein angiography (FA) can be used. The most used classification is the Shields classification, from stage 1 to stage 5. The three classic features that confirm Coats’ diagnosis are exudative retinal detachment, irregularly dilated telangiectatic vessels, and peripheral nonperfusion. Treatment may include observation, laser photocoagulation or cryotherapy, external drainage of the detachment, or vitreoretinal surgery, depending on the stage of the disease. The prognosis depends on the stage of the disease, with the main risk factors for enucleation being elevated intraocular pressure and neovascularization.