Morquio Syndrome is an autosomal recessive lysosomal storage disease. It is one of the subgroup of mucopolysaccharidosis (MPS IV). Anesthesiologist usually deal with patients diagnosed Morquio Syndrome as they require repeated anesthesia administrations for adenoid and tonsil hypertrophia or ortophedic procedures. Cervical instability, myelopathy, thorax deformability, upper and lower airway obstructions may complicate the anesthetic management of patients who will undergo surgical procedures. Besides, generalized organ and tissue involvement leads to increase in mortality and morbidity. Our aim is to report and discuss the anesthetic management of a patient with Morquio Syndrome who undergoes hemiepiphysiodesis operation for bilateral genu valgum in the light of current literature.