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which the enzymes that break down glycosaminoglycans (GAG) are lacking. Glycosaminoglycans accumulate intralysosomally and disfunction in cells, blood vessels, and organs begin to occur. There are 11 different types of MPS according to deficient enzyme.1,2 Mucopolysaccharidosis types have many common characteristics, such as somatic involvement properties and being normal at birth but presenting a chronic and progressive course over time.3,4 Mucopolysaccharidosis type 6, also known as Maroteaux-Lamy syndrome, is a very rare disease; first defined by Maroteaux et al. in 1965. N-acetylgalactosamine-4-sulfatase enzyme (Aryl-

A Rare Disease: Mucopolysaccharidosis Type 6 and Cardiac Involvement: Case Report