Hamartoma is a nodular malformation, with a single or multiple malformations, it is a recognized prenatal developmental abnormality. This benign tumor lesion has the same histological components of normal parenchyma, but these components are arranged haphazardly which disrupts normal function. Hamartomas usually remain connected with the tissue of origin; as is the case with pulmonary hamartoma or the splenic hamartoma. The exact incidence of retinal astrocytic hamartoma (RAH) is not well known, but it is estimated at one case per 100 000 births per year [1]. The astrocytic hamartoma is a neuroglial tissue and produces astrocytes within the optic nerve [2], and consequently it often appears within papillary region.