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.
CONCLUSIONS: 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.