Carpal tunnel syndrome is a condition rarely encountered in infants. We describe five patients with nine involved hands. One was reported earlier. In three cases the cause of carpal tunnel syndrome is a storage disease: one girl with mucolipidosis type III and a boy and a girl with mucopolysaccharidosis type I. The fourth case is a girl presenting with unilateral macrodactyly of the right third and fourth digits. The last case involves a boy with familial carpal tunnel syndrome. We briefly discuss these patients and review the literature since 1989.