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Retinoblastoma is the most common intraocular neoplasm in children. Glial tumor of the retina and optic nerve head are considered to be congenital and are therefore classified as hamartomas. Concurrent occurrence of these tumors in one eye is uncommon and by reviewing the studies, a few cases have been reported. We report a 9 years old boy with eye enucleation and concurrent occurrence of retinoblastoma and astrocytoma in one eye as two separate and different masses. Although retinoblastoma and astrocytoma are two distinct tumors and their concurrent occurrence in one eye is rare, concurrent occurrence of these tumors may suggest differentiation of these two tumors from a neuroectodermal primary cell.