Nonsyndromic inherited dentin defects are distinguished clinically as dentin dysplasia type II (DD-II), dentinogenesis imperfecta type II (DGI-II) and dentinogenesis imperfecta type III (DGI-III)6 and are caused by mutations in DSPP. Sharing a common causative gene suggested that the three clinical conditions simply reflect variations in severity of a single disease7 and are caused by the same pathology8. This evidence concerns the gene DSPP and dentin dysplasia type II.