Cleidocranial dysplasia (CCD) is a genetic disorder inherited in an autosomal dominant pattern. It can cause various craniofacial and dental abnormalities. We report a case of a 20-year-old female patient with a chief complaint of delayed eruption of permanent teeth. She has a medical history of wide fontanelle diagnosed at 16 months. Clinical and radiologic examinations showed multiple remained deciduous teeth and unerupted permanent teeth. In addition, in the posteroanterior cephalogram, the skull showed an inverted pear shape. In the MDCT, we found the patency of anterior fontanelle, incomplete mandibular symphysis and multiple Wormian bones along the temporo-occipital suture. In the chest radiograph, thorax showed narrow cone-shaped and both clavicles were incompletely formed only in the distal part. No mutation of RUNX was observed in the genetic analysis. As the patient wanted treatment with less pain and discomfort, we proceeded orthodontic treatment with extraction of all canines and supernumerary teeth not forced eruption of those. The total treatment duration was 3 years and 3 months. Treatment finished remaining upper and lower deciduous canine and lower both second molars. After 3 years of treatment, tooth alignment and occlusion were maintained well.