Cleidocranial dysplasia (CD) is an autosomal dominant skeletal disorder with characteristic dental findings of numerous supernumerary teeth and noneruption of permanent teeth. This investigation compared the dental development and root lengths of the mandibular first permanent molar in 11 CD patients with those of 22 healthy, normal children matched for race, age and sex. The results showed that children with CD experienced a delay in dental development of approximately 3 years compared with normal children (P < 0.05). In addition, the root lengths of the mandibular first permanent molar were significantly longer than those of the comparison children (17.8 +/- 1.6 mm vs 13.6 +/- 1.2 mm (P < 0.001). This study thus revealed two significant clinical features of CD: 1) severe delay in dental development, and 2) excessive root lengths of mandibular permanent first molars. These features may be important in the pathogenesis of delayed dental eruption observed in this disorder.