The most prominent congenital syndrome is Down syndrome (DS). With the greatest number of births of all chromosomal disorders, it is the most well-known reason of physical as well as intellectual impairment. A comparatively elevated incidence of Down syndrome has been discovered in Saudi Arabia. Only few papers have been undertaken thus far describing the features of face and other oral features of Saudi Down syndrome subjects. To review the available literature on Down syndrome and it's oral effects in Saudi Arabia region. Down syndrome also described as trisomy of chromosome 21. It is a genetic autosomal inherited condition i.e non-sex chromosomal disorder that affects one in six hundred to one in two thousands live births in various populations and is marked by a deficit in physical and intellectual development. Due to the comparative elevated incidence of DS in Saudi Arabia—1 in every 554 live births—DS subjects were specifically chosen for this review of literature. There are now more people with DS as a result of the lowered mortality rate and rising incidence in Saudi Arabia population. The most noticeable craniofacial features are found in DS patients, along with other distinguishing physical and systemic manifestations. Midface deficit, prognathism of mandible, depressed position of nasal bridge, slanting of eyes, folds at the epicanthic region, condition of hypotelorism in ocular area, and strabismus constitute the documented craniofacial characteristics. Muscle hypotonia also affects the perioral muscles, which results in a lowering of the mouth's angle. A comparatively elevated incidence of Down syndrome has been discovered in Saudi Arabia. Only few papers have been undertaken thus far describing the features of face and other oral features of Saudi Down syndrome subjects. It can be concluded that individuals with Down syndrome in Saudi Arabia had a significantly higher incidence of facial as well as cranial features in comparison to the normal healthy counterparts. By using the search algorithms “(Down syndrome and dental caries, periodontal diseases), “(Down syndrome and deformity within oral structures), “(Down Syndrome and problems in occlusion and Craniofacial structures).”The researcher searched the Cochrane Central Systematic Reviews Registry, ISI Web of Science, and Medline without language restriction up to July 2022. Only few papers have been undertaken thus far describing the features of face and other oral features of Saudi Down syndrome subjects. It can be concluded that individuals with Down syndrome in Saudi Arabia had a significantly higher incidence of facial as well as cranial features in comparison to the normal healthy counterparts.