Biliary cast syndrome (BCS) is defined as the necrotic debris filling the intra- or extra-hepatic biliary tracts and forming casts that take the shape of the biliary tree after liver transplantation, thus leading to a series of clinical manifestations. It is an unusual but very serious complication of liver transplantation. A better knowledge of the etiology, pathogenesis and clinical features of BCS may help prevent the occurrence of BCS and improve the survival of post-liver transplantation BCS patients, thus, the authors address the related issues along with the diagnosis and treatment of this condition.