Mucopolysaccharidosis type VI or Maroteaux Lamy syndrome is an autosomal recessive lysosomal storage disorder resulting from a deficiency of arylsulfatase B. Maroteaux et al. first described this disorder as a novel dysostosis associated with increased urinary excretion of chondroitin sulfate in 1963 1. The gene discussed is ARSB; the disease is lysosomal storage disease.