The objective of this chapter is to introduce the Mucopolysaccharidosis. Mucopolysaccharidosis is a group of lesions of generalized skeletal abnormalities characterized by dwarfism affecting the spine1 and limb, visceral abnormalities and evidence of a lysosomal storage disorder. Diagnosis is made by thorough assessment of the clinical and radiographic features, genetic studies, laboratory findings, and determination of the precise enzyme deficiency by urine studies and skin fibroblast cultures. This chapter also discusses differential diagnosis and treatment of mucopolysaccharidosis. Occipitoupper cervical fusion is performed to stabilize the atlantoaxial joint. The clinical findings include progressive cerebral degeneration, visceromegaly and dysostosis multiplex. The infantile is characterized by severe neurological disability hepatosplenomegaly, blindness, and seizures.