Ellis-van Creveld syndrome (EvC), also called chondroectodermal dysplasia, is a rare occurrence inherited as an autosomal recessive disease. Despite the fact that oral manifestations play an important role in the diagnosis criteria for EvC, few detailed reports have been published in the dental literature. This articles presents two siblings with EvC, a boy aged 9 years and a girl aged 7 1/2 years, a product of unaffected first cousin parents. The patients manifests: chondrodysplasia of tubular bones resulting in disproportionate dwarfism, polydactyly and syndactyly of hands and feet, severe dystrophic nails, multiple broad labial frenula with abnormal attachments, congenital missing incisors, anomalous teeth, bilateral partial clefts of the alveolar bone, and malocclusion. Other features noted in either cases are: congenital heart defect, median notch of the upper lip, shovel-shaped incisors and taurodontism. Of the unusual dental findings observed in our patients are talon cusp, reduced crown size, supernumerary tooth, and early eruption of teeth. Because half of the cases with EvC have cardiac malformation, dental treatment must be performed under prophylactic antibiotic coverage. Dentists play an important role in early diagnosis and control of dental problem of this condition.