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Ellis-Van Creveld syndrome is a rare autosomal recessive disorder which can be diagnosed at an early stage based on various clinical features and oral manifestations. The aim and objective of this article is to conduct a brief literature review on oral manifestations, diagnostic criteria and treatment protocol of Ellis-Van Creveld Syndrome and also a case study of 7-year-old boy is reported. Two databases were used and totally 261 articles are retrieved, out of which only 16 articles were selected after applying inclusion & exclusion criteria. Results concluded that Dentists should be aware of the features of this syndrome for making early diagnosis and facilitate multidisciplinary treatment.