Ellis-van Creveld syndrome also known as chondroectodermal dysplasia or mesoectodermal dysplasia; a rare genetic disorder of the skeletal dysplasia. 'Six-fingered dwarfism' (digital integer deficiency) was an alternative designation used for this condition when it was being studied in the Amish. It is characterized by short-limb dwarfism, polydactyly, malformation of the bones of the wrist, dystrophy of the fingernails, partial hare-lip, cardiac malformation and often prenatal eruption of the teeth. A typical case of Ellis-van Creveld syndrome is reported in the present article. How to cite this article: Singh S, Arya V, Daniel MJ, Vasudevan V. Ellis-van Creveld Syndrome: A Case Report . Int J Clin Pediatr Dent 2012;5(1):72-74.