This text presents a major group of causes of end-stage chronic liver disease. There has been great scientific interest in these disorders in recent years, so this text evaluates treatment modalities in old and even new cholestatic diseases. Since secondary malnutrition plays an important role in inducing multiorgan morbidity in these patients, nutritional and metabolic aspects need better understanding. The influence of disease and nutrition-related problems on liver transplantation as the ultimate therapy of cholestatic disorders is also of current interest.