Ellis Van Creveld syndrome (EVC) is a rare genetic disorder with autosomal recessive inheritance, seen more amongst the Amish population of Pennsylvania in USA with an incidence of 1:2,44,000.” Six fingered dwarfisms” was another name given to this syndrome. This syndrome presents with four clinical characteristic features of ectodermal dysplasia, congenital heart disease, bilateral postaxial polydactyly and nail or teeth hypoplasia. A typical case of a 13-month-old child is present in this article Introduction: EVC syndrome described as chondroectodermal dysplasia; a rare genetic disorder of skeletal dysplasia. It’s a single gene disorder autosomal recessively inherited due to mutations in EVC 1 and 2 genes located on the same chromosome 4p16. In 1940, the first case was described by Richard W.B. Ellis of Edinburgh and Simon Van Creveld in Amsterdam1. It is an uncommon syndrome with a prevalence of seven per 1,000,000 cases2 . There is an equal predilection in males and females. It is more seen in people who are Amish of Lancaster country, Pennsylvania and in the Western Australia. It presents characteristically with the tetrad of (a) Chondroectodermal dysplasia (disproportionate dwarfism with short limbs and longer trunk), (b) bilateral postaxial polydactyly, (c) nail hypoplasia, dystrophic nails, malformed teeth and (d) congenital cardiac malformations, most commonly present as single atrium followed by septal defects 3