Little attention in the dental literature has been given to the dental characteristics of patients with the Wolf-Hirschhorn Syndrome (WHS). The syndrome is caused by deletions of the terminal portion of the short arm of chromosome 4. This case report provides information on dental anomalies noted in a child with WHS. The dental findings include agenesis of multiple permanent teeth, particularly premolars and molars, taurodontism, and over-retained primary teeth. This syndrome exhibits variable clinical expressivity, possibly due to the extent and the specific locus of the chromosomal deletion. Further studies are required to obtain a clearer view of the clinical oral/dental manifestations of this syndrome.
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