Ellis van crevald syndrome or chondroectodermal dysplasia is known to be a rare genetic disorder first described by Richard W.B Ellis & Simon Van crevald in 1940. This syndrome comprises tetrad of chondrodysplasia, bilateral postaxial polydactyly of the hands, ectodermal dysplasia & congenital heart defects.
The orofacial manifestation includes dental malformations, hypodontia, multiple gingivolabial musculo-fibrous frenulae, notching of the lower alveolar process, fusion of the upper lip gingival mucosal margin. This paper includes a case report of a young female patient having similar characteristic features and orofacial manifestations suggestive of this syndrome and its management.