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¹GATA Göz Hastalıkları AD.
²GATA Genetik AD. Ophthalmic, systemic findings and laboratory investigation results are presented in 20 and 16 years old siblings and 1.5 years old male nephew of a family with the characteristics of Hunter syndrome. Ring type retinitis pigmentosa found in one patient is an ophthalmic finding that is unrelated to the classical data about the syndrome. We here report three cases of Hunter syndrome incidence of which is 1/300.000 and they are the first presentations in Turkish literature as far to our knowledge. Keywords : Mucopolysaccharidosis, Hunter Syndrome, Ophthalmic Findings