Introduction: Mucopolysacchridosis is a lysosomal storage disorder due to deficiency of lysosomal hydrolase class of  enzymes mucopolysaccharidases. It is rare, inherited autosomal recessive disorder results from abnormal accumulation of undegraded GAGs in lysosome of various tissues. Patient Info: Skeletal abnormalities, macroglossia, facial dysmorphia, Scoliosis, excessive salivation are few of the characteristic symptoms which make one to suspect this disorder. We present 9 year old male child having macroglossia and hyper salivation. Diagnosis: He was diagnosed as a case of Mucopolysacchridosis after Berry’s Toluidine blue spot test and urine electrophoresis results.                                                              Keywords: Mucopolysacchridosis, Glycosaminoglycan, Lysosomal storage disorder,Electrophoresis, metabolic disease, screening tests, Carbohydrate, Heparan sulphate, Dermatan sulphate, Mucopolysaccharide,