Introduction. Cleidocranial dysostosis is a syndrome defined by three characteristic findings: clavicular aplasia,  retarded cranial ossification, and autosomic dominant hereditary transmission, with completed penetrance and full expression.  However, the diagnosis cannot only be made based on those finding, because the polymorphism and extension of the lesions of  this disease is important. Therefore, in this disease we can see upset in the second teething, short stature or dwarf, persistence  of the biconvex appearance of vertebral body, bone hypoplasic iliac, retarded pubis branch ossification, wedge shape distals  phalanges or with braquimesophalangia of the forefinger and fifth finger. Clinical case. We describe a 20 years old man, with  cleidocranial dysostosis, without familiar antecedent (probable mutation), that come to our center for treatment of denture  pathology with disabled eating, because anomalous distribution and eruption. He had clavicle agenesis, cranial ossification  upset with wormians bones, vertebral bodies biconvex, superior maxillary hypoplasic, and dental packed in the superior  maxillary and jawbone. Conclusions. Cleidocranial dysostosis is a hereditary disease, which can be of spontaneous apparition  (mutation), has a grand polymorphism, affect the osseous development, predominate in the middle line membranous bone and  is an entity of radiologic diagnosis [ Introduction. Cleidocranial dysostosis is a syndrome defined by three characteristic findings: clavicular aplasia,  retarded cranial ossification, and autosomic dominant hereditary transmission, with completed penetrance and full expression.  However, the diagnosis cannot only be made based on those finding, because the polymorphism and extension of the lesions of  this disease is important. Therefore, in this disease we can see upset in the second teething, short stature or dwarf, persistence  of the biconvex appearance of vertebral body, bone hypoplasic iliac, retarded pubis branch ossification, wedge shape distals  phalanges or with braquimesophalangia of the forefinger and fifth finger. Clinical case. We describe a 20 years old man, with  cleidocranial dysostosis, without familiar antecedent (probable mutation), that come to our center for treatment of denture  pathology with disabled eating, because anomalous distribution and eruption. He had clavicle agenesis, cranial ossification  upset with wormians bones, vertebral bodies biconvex, superior maxillary hypoplasic, and dental packed in the superior  maxillary and jawbone. Conclusions. Cleidocranial dysostosis is a hereditary disease, which can be of spontaneous apparition  (mutation), has a grand polymorphism, affect the osseous development, predominate in the middle line membranous bone and  is an entity of radiologic diagnosis