A case of chondroectodermal dysplasia, by Ellis, van Creveld, is described manifested both by changes in oral cavity and in the skeleton. The patient is a nine-year-old girl with shallow vestibulum oris, supernumerary mucosal plicae in the frontal part of the vestibulum oris, oligodontia and involvement of tooth shape. Among other symptoms there were polydactylia (corrected by surgery), club-shaped fingers and koilonoychia. Bone X-ray showed alterations typical for this type of enchondral dysplasia. This case can, in our opinion, be managed only by prosthesis. This syndrome, fortunately, is but rarely seen.

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