AbstractPrimary cilia are microtubule‐based organelles that serve as hubs for the transduction of various developmental signaling pathways includingHedgehog, Wnt, FGF, and PDGF. Ciliary dysfunction contributes to a range of disorders, collectively known as the ciliopathies. Recently, interest has grown in these syndromes, particularly among craniofacial biologists, as many known and putative ciliopathies have severe craniofacial defects. Herein we discuss the current understanding of ciliary biology and craniofacial development in an attempt to gain insight into the molecular etiology for craniofacial ciliopathies, and uncover a characteristic ciliopathic craniofacial gestalt. genesis 49:231–246, 2011. © 2011 Wiley‐Liss, Inc.