Carpal tunnel syndrome in children is uncommon. Mucopolysaccharidosis is the most common cause of carpal tunnel syndrome in this age group. With new treatment modalities available for mucopolysaccaridoses, the prognosis of the disease has greatly improved. The musculoskeletal manifestations including carpal tunnel syndrome now assume more importance. Early diagnosis and treatment of carpal tunnel syndrome in these children are important to achieve a good outcome. The authors present their experience in the management of children with mucopolysaccharidosis and carpal tunnel syndrome.