The purpose of this study was to analyze craniofacial growth in cleidocranial dysplasia (CCD) with emphasis on bone remodeling, mandibular condylar growth, and tooth eruption. Skull radiographs of 22 CCD children followed longitudinally were examined. The span of time investigated varied for each patient, but the period from 5 years to adult age was well covered. Five patients had metallic implants inserted in the jaws; the implants enabled detailed analysis of bone remodeling, jaw rotation, and tooth eruption. In the calvaria, an open anterior fontanelle area persisted in most cases but decreased with age. A frontal sinus failed to develop or was diminutive in all cases but one. In the cranial base, the size increase of the sella turcica was reduced as a result of modest resorption at the floor and the posterior wall; the clivus was flexed in most cases, but the flexion remained stable during the observation period. Growth in maxillary height was severely reduced, primarily because resorptive lowering of the nasal floor was minimal. The amount of bone apposition on the orbital floor and the alveolar process was smaller than expected. Condylar growth direction was vertical giving rise to a forward rotation of the mandible in relation to the anterior cranial base. The expected resorptive remodeling below the mandibular angle and anteriorly on the ramus was negligible. The low maxilla, in combination with a marked forward mandibular rotation in cases with unstable occlusion as a consequence of eruption problems in the permanent dentition, gave rise to a diminished anterior facial height.(ABSTRACT TRUNCATED AT 250 WORDS)