Ellis Van Creveld syndrome (EVC) or chondroectodermal dysplasia is a rare congenital disorder of autosomal inheritance. It is commonly seen in Amish population of Pennslyvania in USA with the prevalence of 1/5,000 live births. In non- amish population the prevalence is 7/1,000,000 live births. It is caused by mutation in the EVC gene located on chromosome 4p16 and also mutation of EVC2 gene located close to EVC gene in a head to head configuration. It is characterized by bilateral postaxial polydactyly of hands, dysplasia of teeth and nails, short limbed dwarfism and congenital heart disease. This case report describes a 19 year old year Indian female born to a consanguineous marriage with the classical features of Ellis Van Creveld Syndrome with complete AV canal defect with relatively asymptomatic childhood with classical goose neck deformity in left ventricular angiogram