Describe the sonographic findings in assessment of the chondroectodermal dysplasia. Descriptive study of two cases with chondroectodermal dysplasia diagnosed in our center. Maternal fetal medicine specialists performed the prenatal ultrasound and postnatal follow-up was obtained in all cases. The two cases were presented to the Prenatal Diagnosis Committee. The skeletal dysplasias correspond to 12% of the cases evaluated in our unit between 2003 and 2006. We had two cases of Ellis-van Creveld syndrome in 23 520 live births, with incidence 1/11 760. In our cases we had CNS, facial, heart and limb anomalies. We present the images in ultrasound and postnatal correlation. Anomalies associated to the Ellis-van Creveld syndrome involve the three germinal lines: ectodermic dysplasia (affects tooth, nails, hair and lips), mesodermal dysplasia (affects bones, heart and kidneys) and endodermic dysplasia (affects liver and lungs). For the diagnosis, it is necessary to meet three of the four criteria: ectodermic dysplasia, chondro-dysplasia, postaxial polydactyly and congenital heart disease. The prenatal assessment of suspected skeletal dysplasias using ultrasound presents great challenges both in assigning an accurate prognosis. Our experience shows that the adequate knowledge of features of this type of skeletal dysplasia and sequential ultrasound examination raise the possibility of accurate diagnosis.