health care professionals to prevent from further complications. CASE REPORT Figure 1: Missing lower incisors and upper laterals. ABSTRACTEllis-van Creveld syndrome is an extremely rare congenital genetic disorder having autosomal recessive inheritance. The characteristic features of this syndrome are bilateral postaxial polydactyly, acromesomelic dwarfism, ectodermal dysplasia affecting nails, congenital cardiac malformation, edentulous maxillary and mandibular incisors, nonappearance of mucobuccal fold, congenitally missing teeth, slight serrations of the alveolar ridge and multiple small alveolar notches. The present case describes the oral manifestations of the patient which leads to a perfect diagnosis of this syndrome. Ellis-van Creveld syndrome requires a multidisciplinary management and, hence the dental surgeons play an important role in these cases.