Aim A systematic literature review on oral and craniofacial manifestations of Ellis-Van Creveld syndrome was performed. Methods From 2 databases were selected 74 articles using as key words "Ellis-Van Creveld" AND "Oral" OR "Craniofacial" OR "Dental" OR "Malocclusion". Prisma protocol was used to create an eligible list for the screening. Data were collected in a table to compare the clinical aspects found. Results From the first research emerged 350 articles, and only 72 of them were selected. Conclusion Through this analysis oral and cranio-facial manifestations of Ellis-Van Creveld syndrome were pointed out. Management of Ellis-Van Creveld syndrome requires a multidisciplinary approach involving different clinicians; dentists play an important role in early diagnosis and treatment of the patients
