Ellis-van Creveld syndrome or chondroectodermal dysplasia is a rare autosomal recessive disorder. It was first described by Ellis and van Creveld in 1940. It is a chondral and ectodermal dysplasia characterized by short ribs, postaxial polydactyly, growth retardation, and ectodermal (dysplastic teeth and nails) and congenital heart defects. Approximately 60% of patients have a cardiac defect, most commonly an atrial septal defect, often with a single atrium. In this article, we presented a case of Ellis-van Creveld syndrome associated with single atrium as well as rare distal limb shortening.