Abstract: Interstitial cell of Cajal (ICC) is a kind of special interstitial cell in the gastrointestinal tract, which has close connection with the smooth muscle cells and neuron cells. It is involved in gastrointestinal motility and nerve signal transduction and is considered as the pacemaker cell of slow wave of gastrointestinal tract. ICC which exists in the nongastrointestinal organs such as biliary tract, urethra, bladder and so on is named interstitial Cajallike cell (ICLC). This article will review the progress in the research of ICLC in the biliary system in terms of their morphology, identification, classification, function, distribution and related biliary diseases.