Abstract
Ellis-van Creveld (EVC) syndrome or chondroectodermal dysplasia is a rare autosomal recessive disease, with an incidence of 1:244,000 for the general population. The exact prevalence of this syndrome is unknown but believed to be a genetic chromosomal disorder with a higher incidence in women. EVC syndrome consists of a tetrad of principal features: chondroectodermal dysplasia, polydactyly, congenital heart defects, and hypoplastic nails and teeth. In this syndrome, many other organs can be compromised with multiple oral manifestations of hard and soft tissues, but dental literature on the subject is scarce. Hence, we report a case of a patient, visiting Maratha Mandal's NGH Institute of Dental Sciences and Research Centre, Belgaum, seeking dental treatment presenting the typical features of EVC syndrome.
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