Coats disease is an idiopathic nonhereditary condition retinal vascular disorder characterized by retinal telangiectasia with intraretinal and/ or subretinal exudation. Unilateral involvement in young males is the typical presentation with most cases being diagnosed in the first and second decade of life and, if untreated, can lead to total retinal detachment and secondary glaucoma, sometimes requiring enucleation. The most common presenting complaints include decreased vision, strabismus, and leukocoria. This article describes the clinical features, pathophysiology, classification and staging, complications, differential diagnosis, and the management of Coats disease.