Mucopolysaccharidosis type VI (MPS VI), or Maroteaux-Lamy syndrome, is an autosomal recessive genetic and lysosomal disorder resulting from mutation of the ARSB gene that encodes lysosomal enzyme N-acetylgalactosamine-4-sulfatase (arylsulfatase B or ARSB, EC 3.1.6.12) [1,2], with a commonly predicted incidence rate of 1 per 100,000 live births [3]. The gene discussed is ARSB; the disease is lysosomal storage disease.