Dentocraniofacial structure with ectodermal dysplasia, showing complete anodontia of permanent teeth, was examined. Except for a slight decrease of the sweat pore count, the patient was free of signs of ectodermal dysplasia, other than odontoplasia. Furthermore, her family history had no record of hypodontia or anodontia. We attempted to clarify the role of tooth growth in the dentocraniofacial development. Cephalometric analyses revealed the extreme limitation of the maxillary and vertical growth of the lower face, and a retracted mandibular alveolus front. We concluded that the role of tooth growth was significant in the development of the maxillary and alveolar bone, but not in the development of the mandible.