Tehran University of Medical Sciences Journal of Craniomaxillofacial Research 2345-5489 1 3-4 2015 10 12 66 68 EN Alireza Parhiz Department of Oral and Maxillofacial Surgery, Dental School, and Craniomaxillofacial Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran 2015 10 12 2015 10 12 Klinefelter syndrome includes a group of chromosomal disorders with at least one  additional  X  chromosome  in  male  karyotype  (46,XY).  Up  to  now, different dental manifestations such as taurodontism, congenital absence of permanent teeth, shovel incisors, occlusal anomalies and increased permanent tooth size have been reported. A case of Klinefelter syndrome with very rare dental features is presented. https://jcr.tums.ac.ir/index.php/jcr/article/view/17 https://jcr.tums.ac.ir/index.php/jcr/article/download/17/63