Non-syndromic dentin defects categorized as dentin dysplasia type II (DD-II), dentinogenesis imperfecta type II (DGI-II) and type III (DGI-III) are generally caused by dominant mutations in dentin sialophosphoprotein (DSPP), which encodes the most abundant non-collagenous matrix component of dentin [3]. Here, DSPP is linked to dentinogenesis imperfecta.