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in the Hunter Syndrome or Mucopolysaccharidosis type II (MPS II), a recessive genetic disorder linked to the X chromosome, there is a deficiency or absence of iduronate-2-sulfatase enzyme responsible for the catabolism of Heparan sulfate and dermatan sulfate, causing, thus, a deposit of these mucopolysaccharides. These are found in skin, blood vessels, heart, heart valves, lungs, arteries and cellular surfaces, having their intralysosomal accumulation and several morphological and physiological consequences of gradual worsening. For treatment of patients with Hunter Syndrome is provided Enzyme Replacement Therapy (ERT), consisting of the intravenous administration of the deficient enzyme. This study aims to analyze the evolution of pre- and post ERT frame. Method: for this analysis we used the case report of the patient in question. Results: it was found a patient with classic clinical manifestations of MPS II, which evolved from the ERT with marked improvement in the clinical picture from advances in respiratory status, pubertal development, reduction of joint stiffness and platelet stabilization. Conclusion: it was possible to conclude that it is extremely important

Manifestações clínicas e terapia de reposição enzimática da Síndrome de Hunter: relato de caso