Choroidal osteoma (CO) is a rare, benign, and usually unilateral intraocular tumor composed of mature bone affecting the choroid. It appears as slightly elevated, yellowish-white, or orange choroidal mass with well-defined borders. It is commonly encountered in young female adults. Here, we report findings of a 4-week-old premature baby with CO in her left eye which was detected during a routine examination for “retinopathy of prematurity.” We believe that this case is the youngest patient reported with CO which showing that this pathology can be encountered even in a newborn.