Oral manifestations of Morquio's syndrome

The clinical and laboratory findings in two siblings affected with Morquio's syndrome are described. The oral findings, consisting of disturbances in the structure of the enamel in both deciduous and permanent teeth, resemble those of amelogenesis imperfecta, Type 1. Preformed crowns are recommended as the initial treatment, in order to avoid loss of vertical height. The dentist's familiarity with the oral manifestations of Morquio's disease may contribute to the early detection of this condition.