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By histopathology, the diagnosis of XGO was rendered. The preoperative diagnosis of XGO can very challenging therefore the analysis mainly hinges on histopathologic assessment. Adequate pathologic sampling is essential to rule out the chance of co-existing testicular neoplasms.Mucopolysaccharidosis type I (MPS I) is an inherited metabolic disorder caused by lack of alpha-L-iduronidase (IDUA), causing accumulation of heparan and dermatan sulfate glycosamin