Morquio syndrome is one of the mucopolysaccharidoses. Glycosaminoglycans accumulate within the cells, leading to many systemic alterations. Alterations in connective tissue and cartilage ground substance result in abnormal formation and growth of the skeletal system. The major orthopedic manifestations include shortening of the trunk and limbs, spinal curvature, odontoid hypoplasia with upper cervical instability, and lower-limb alignment problems. The management of the orthopedic manifestations of this syndrome requires a broader knowledge of the genetic, metabolic, and systemic effects of the metabolic alterations.