Introduction: Hurler syndrome is an autosomal recessive inborn error of metabolism, resulting from deficiency of a-liduronidase, a catabolic mucopolysaccharidase, which leads to excessive systemic storage of the glycosaminoglycans (GAGs) heparan sulphate and dermatan sulphate [1]. Affected children may appear normal at birth. However, progression of the disease results in characteristic facial changes, hepatosplenomegaly cardiomyopathy, major skeletal abnormalities and CNS damage, resulting in severe disability, intellectual regression and death, usually by the age of 10 years. We report the case of a 7-year-old girl who was consulting for a visual impairment with photophobia. The ophthalmologic examination showed corneal opacities associated with bilateral papilled edema with an eye tone of 18mmHG for the eye right and 19 mmHG for the left eye. The abdominal examination finds a protruding abdomen with a hepato splenomegaly. Cardiovascular examination with doppler echo objective thickening mitro-aortic and tricuspid valves. The diagnosis was evoked before facial dysmorphism and then confirmed biologically (MPStype I-H). A bilateral trabeculectomy was performed while waiting for a marrow allograft. The early diagnosis of MPS, before the formation of neurological deficits, has become essential, since the treatment can stop the evolution. Thus, a better knowledge of the clinical picture by ophthalmologists could improve the prognosis