Ogunbiyi, A.Adeyinka, A. O.Ogah, S. O.Baiyeroju, A. M.2018-10-162018-10-162006-06WAJM 25(2), pp. 169-172http://ir.library.ui.edu.ng/handle/123456789/2733FrenchHunter syndrome is one of the mucopolysaccharidosis, which are a rare group of genetic diseases, It is due to a deficiency in the enzyme Iduronate 2-sulphatase. This in turn leads to the accumulation of glycosaminoglycans, dermatan and heparan sulphate. The intra and extracellular accumulation of this substances lead to multisystemic organ abnormality. We present a patient with Hunter syndrome who presented with involvement of the skin, cardiovascular system, the eyes and musculoskeletal system. We have also included a literature review. As far as we know, it is the first case reported in a Nigerian.en-USHunter syndrome,Complicotions,Nigerian.Article