Morquio syndrome is a rare autosomal recessive inherited disease of lysosomal storage defect associated with multiorgan involvement and symptoms are seen due to excessive accumulation of Glucose-aminoglycans in the affected sites. Its characteristic features are extremely abnormal gait, short stature, short neck, and macrocephaly which can be accompanied with high level of GAG KS in urine during urine analysis and atlantoaxial subluxation and other characteristic radiographic features for proper diagnosis. Many oral manifestations are associated with this disease like hypoplastic enamel, abnormal tooth anatomy, macroglossia, temporomandibular joint defect, therefore dental intervention is necessary at an early stage but there are many features which might interfere in normal dental treatment, that’s why detailed knowledge about this syndrome will help dentists to handle these patients more efficiently.

Keywords : Morquio Syndrome, Lysosomal Defect, Mucopolysaccharidosis, Multiorgan Involvement, Oral Manifestations.