Among the lysosomal storage diseases which arise due to inherited deficiencies of lysosomal enzymes, mucopolysaccharidoses are important for orthopedic surgeons due to their severe and vast skeletal manifestations. Short stature, cervical instability, stenosis of the craniocervical junction, kyphosis, carpal tunnel syndrome, trigger finger, hip dysplasia and genu valgum are the cardinal orthopedic problems in mucopolysaccharidoses. These problems usually require surgical interventions. Follow-up and treatment of patients with mucopolysaccharidosis must be managed by multidisciplinary teams which include orthopedic surgeons.