The interstitial cells of Cajal (ICCs) are a population of cells in the gastrointestinal tract which have a role in the control of gut motility. A comprehensive review of the scientific literature was undertaken to assess current understanding of the morphology, structure, identification, distribution, development and function of these cells. ICCs have an important role in the control of gut motility. Experimental evidence from animal studies suggests roles as pacemakers and coordinators of gut motor activity, and as intermediaries in the neural control of motility. With an increasing understanding of the distribution and behaviour of these cells in the healthy or diseased human gastrointestinal tract, there is the potential to develop novel therapeutic approaches to diseases that have gut dysmotility as a contributory factor.