Clincal and biochemical manifestation were evaluated in 30 patients clinically suspected of mucopolysaccharidosis. Clinicall, Echocardiography revealed abnormal findings in 23 patients (93%) and upper airway obstructin in 21 patients (70%) and hepato-splenomegaly in 16 patients (53%) corneal opacities were found in 15 patients (43%) whil hearing loss in 7 after electrophoresis analysis of urinary glycoaminoglycans (GAG) 24 patients were high for GAG level and 6 patients were normal GAG level . The enzyme activity level for these 24 affected patients were measured ; a-iduronidase level was low in 14 patients (58%) and Galactose-6- phosphate level was low in 10 patients (41%) .