Five sporadic cases of cleidocranial dysostosis are described. Besides the classical findings, the clinical and roentgenological evaluation showed bilateral macrodactily of the second toe, aplasia of the 12th rib and severe acetabular dysplasia. This features had not been previously reported in medical literature. The importance of an early diagnosis is stressed for adequate orthopedic and odontologic management, as well as for opportune genetic counseling.