Coats disease is characterized by idiopathic congenital retinal telangiectasis and exudative retinopathy. Mostly young males are affected. The disease is usually unilateral. Visual prognosis varies from full visual acuity to blindness according to the extent of retinal exudation. Retinoblastoma is a major differential diagnosis particularly in small children but other causes of exudative retinopathy must also be considered. Treatment aims include obliteration of the telangiectasis in order to resolve retinal exudation and achieve reattachment of the retina. Therapeutic options comprise laser coagulation or cryotherapy, drainage of subretinal fluid, buckling surgery and pars plana vitrectomy. In more advanced cases with therapy-resistant secondary glaucoma enucleation may be required. More recently, intravitreal administration of VEGF inhibitors has become an option.