Chondroectodermal Dysplasia is a disease complex consisting of bilateral manual polydactyly, chondrodysplasia of long bones resulting in acromelic dwarfism, hydroitic ectodermal dysplasia affecting principally the nails, teeth and hair and congenital heart malformations. It is necessary to identify this disease at its early stage in order to render prompt treatment. The oral manifestations are characteristic for this particular disease so that a dental surgeon can identify this condition and refer the case to a cardiologist and orthopedician for corrective surgeries. In this article, a case of a 2 1/2-year old child with chondro-ectodermal dysplasia is presented along with radiographic investigations and treatment plan. MEDLINE® is the source for the citation and abstract of this record.