Coats disease is a rare retinal disease. It is characterized by abnormal dilatation of retinal capillaries (telangiectasia) and increased vascular permeability (intraretinal and subretinal exudation).In an advanced stage of the disease, the patient can have exudative retinal detachment where the retina detaches from its normal attachment. In some cases, there will be an increase in intraocular pressure if not treated on time.It has a unilateral presentation in most of the cases. This article describes about a four-year-old boy who presented with retinal detachment due to Coats disease. The patient was managed effectively with external subretinal fluid drainage along with laser and cryotherapy.It is essential to rule out aggressive eye cancer  (retinoblastoma ) as both can have a similar presentation.