Mucopolysaccharidoses are uncommon hereditary disorders leading to organ dysfunction and anatomic abnormalities. They are accompanied by increased risk of perioperative and postoperative complications. Mucopolysaccharidoses (MPS) are a group of syndromes in which there is an inherited lack of a lysosomal enzyme. Widespread, progressive mucopolysaccharide tissue deposition may cause both organ dysfunction and anatomical abnormalities. The Maroteaux-Lamy syndrome (MPS VI) is a very rare mucopolysaccharidosis with variable severity of somatic and possible cervical spine involvement.