I read with interest the letter to the editor by Digilio et al. [1]. Since the syndrome of hexodactily and single atrium that I have described [2] was partly misinterpreted, I take the liberty to explain the situation. In the case that I reported, no chonrodysplastic changes were noted in the pelvis and tubular bones, nor were there any findings related to ectodermal dysplasia (nails, hair, teeth, gums, and labial mucosa), which are the salient features of the Ellis–van Creveld syndrome [3]. The authors want to relate the syndrome that I have described to the Ellis–van Creveld syndrome and they point out as proof the specific occurrence of AV canal defect and/or single atrium in all the reported cases of Ellis–van Creveld syndrome. However, although single atrium and endocardial cushion defects are the most common anomalies, and persisting left superior vena cava, anomalous pulmonary venous drainage, and PDA may accompany these defects in the Ellis–van Creveld syndrome, the incidence is about 65% [3]. I believe that, if no chondroectodermal dysplastic features are present, the combination of postaxial hexodactily with single atrium should not be considered a variable phenotype of the Ellis–van Creveld syndrome.