Although the expression of cilia on chondrocytes was described over 40 years ago, the importance of this organelle in skeletal development and maintenance has only recently been recognized. Primary cilia are found on most mammalian cells and have been shown to play a role in chemosensation and mechanosensation. A growing number of human pleiotropic syndromes have been shown to be associated with ciliary or basal body dysfunction. Skeletal phenotypes, including alterations in limb patterning, endochondral bone formation, craniofacial development, and dentition, have been described in several of these syndromes. Additional insights into the potential roles and mechanisms of cilia action in the mammalian skeleton have been provided by research in model organisms including mouse and zebrafish. In this article we describe what is currently known about the localization of cilia in the skeleton as well as the roles and underlying molecular mechanisms of cilia in skeletal development. © 2008 Elsevier Inc. All rights reserved.