IntroductionEllis‐van Creveld (EVC) syndrome is an autosomal recessive disorder predominantly characterized by a disproportionate dwarfism, ectodermal dysplasia, postaxial polydactyly, and congenital heart malformations and pulmonary hypoplasia.ObjectiveIn this article, we hereby present a case of a 6‐year‐old Brazilian boy with EVC syndrome who presented a rare oral lesion as well as a remarkable number of classical and uncommon oral and dental features.Case ReportClinical and radiographic examination revealed multiple enamel hypoplasia, teeth agenesis, conical teeth, lower canine rotation, bilateral posterior crossbite, taurodontism of deciduous and permanent molars and delayed tooth eruption, dental caries, and absent vestibular sulcus. Additionally, a whitish lobulated nodule located in the alveolar ridge in the anterior region of the mandible was noted. Anatomopathological examination was compatible with the diagnosis of peripheral odontogenic fibroma (POF). In a 10‐month clinical follow‐up, no signs of recurrence were observed.ConclusionIn view of the characteristic oral findings of EVC syndrome and the potential for recurrence of POF, the pediatric dentist plays an essential role in clinical follow‐up, planning and preventive, and rehabilitative treatment.

Ellis–van Creveld syndrome is an autosomal recessive disorder characterized by a tetrad of disproportionate dwarfism, ectodermal dysplasia, postaxial polydactyly, and congenital heart malformations. In this article, we hereby present a case of a 6-year-old Brazilian boy with Ellis–van Creveld syndrome who presented with a remarkable number of classical oral and dental features and uncommon findings such as taurodontism. Clinical examination revealed multiple enamel hypoplasia, absent vestibular sulcus, alveolar serrations in the maxilla anterior region, missing teeth, conical teeth, lower canine rotation, bilateral posterior crossbite, dental caries, and a nodule. Radiographically were observed teeth agenesis, taurodontism of deciduous and permanent molars, and delayed tooth eruption. Clinical and radiographic findings may be present from birth and the pediatric dentist has a fundamental role in the early diagnosis of Ellis–van Creveld syndrome, as well as oral problems prevention, rehabilitation, and aesthetic interventions.