This chapter describes vanishing bile duct syndrome. Group of acquired disorders associated with progressive destruction and disappearance of the intrahepatic bile ducts and, ultimately, cholestasis. Ductopenia histologic descriptive term for the loss of small intrahepatic bile ducts from any cause. Intrahepatic ductal system includes all ducts proximal to the right and left hepatic ducts. They are divided into two parts such as intrahepatic large bile ducts and intrahepatic small bile ducts. Clinical features of vanishing bile duct syndrome are fatigue, anorexia, abdominal pain, and weight loss.