Various drugs of toxins have been implicated in the development of a particular form of liver damage predominantly involving the bile ducts. Such liver toxicity is often associated with a clinical picture of prolonged cholestasis and may even evolve in rare instances, into the full picture of the vanishing bile duct syndrome, eventually complicated with biliary cirrhosis. Drug and toxins potentially responsible for bile duct injury are reviewed as well as the characteristics of its clinical presentation. The pathophysiologic aspects of the syndrome are also reviewed including recent data, which are strongly in favor of the role of a genetic predisposition.