Two cases of Papillon-Lefevre syndrome have been seen in the same family. The characteristic features of the syndrome are shown to be present in both children and the main characteristics are hyperkeratosis of the palms of the hands and soles of the feet; destruction of the periodontium of the deciduous teeth with subsequent premature loss of the teeth; and destruction of the periodontium of the permanent teeth and the premature loss of these teeth usually before 16 years of age.