Tehran University of Medical Sciences Frontiers in Dentistry 2676-296X 11 3 2014 06 15 361 4 Dana Tahririan Resident, Department of Pediatric Dentistry, Faculty of Dentistry, Isfahan University of Medical Sciences, Isfahan, Iran. Alireza Eshghi Associate Professor of Pediatric Dentistry, Isfahan University of Medical Sciences, Isfahan, Iran. Pirooz Givehchian Resident, Department of Prosthodontics, Faculty of Dentistry, Isfahan University of Medical Sciences, Isfahan, Iran. Mohammad Ali Tahririan Assistant Professor of Orthopedics, Isfahan University of Medical Science, Isfahan, Iran. 2015 10 16 Chondroectodermal dysplasia (Ellis-Van Creveld syndrome) is a rare autosomal recessive congenital abnormality. This syndrome is characterized by a spectrum of clinical findings, among which chondrodystrophy, polydactyly, ectodermal dysplasia, and congenital cardiac anomalies are the most common. It is imperative to not overlook the cardiac complications in patients with this syndrome during dental procedures. The case presented here, although quite rare, was detected under normal conditions and can be alarming for dental care providers. Clinical reports outline the classical and unusual oral and dental manifestations, which help health care providers diagnose chondroectodermal dysplasia, and refer patients with this syndrome to appropriate health care professionals to receive treatment to prevent further cardiac complications and bone deformities. https://fid.tums.ac.ir/index.php/fid/article/view/440 https://fid.tums.ac.ir/index.php/fid/article/download/440/416