Two babies presenting with unilateral leukocoria were found to have total retinal detachment in one eye, associated with subretinal yellowish-white deposits. As no calcification was found and subretinal fluid aspiration yielded many fat-laden macrophages without any evidence of retinoblastoma cells in addition to a high level of cholesterol, the difinitive diagnosis of Coats' disease was made. Subsequently, retinal detachment surgery and cryotherapy were performed, in an attempt to preserve the affected eye.