Cleft lip and/or palate are among the most common birth defects in the world. The prevalence of these conditions varies considerably across geographic areas and ethnic groups. Objective: The aim of this study was to carry out a systematic review and appraisal of the literature on the prevalence of cleft lip and/or palate in Saudi Arabia and comparable Middle Eastern countries. Materials and methods: All published articles on orofacial clefts (OFC) in Saudi Arabia and its bordering countries in the Middle East with similar and comparable population characteristics were reviewed in July 2010. Results: After reviewing the articles, only eight matched the inclusion criteria. Three studies were carried out in two regions in Saudi Arabia (Riyadh and Al-Qaseem). The other five studies were set in Dubai, Oman, and Jordan. The prevalence of cleft lip and/or palate reported in these studies varied greatly from 0.3 to 2.4 per 1000 live births. The birth prevalence of orofacial clefts in males was reported to be higher than in females. The isolated cleft palate prevalence was reported to be higher in females in most of the studies. Conclusion: The eightfold variation in the prevalence of orofacial clefts between highest and lowest prevalence is likely to be due, at least in part, to problems with ascertainment, but there may also be underlying genetic or environmental factors that require further investigation.