Diseases of the bile ducts encompass a wide range of disorders. These include those disorders primarily affecting extra and intrahepatic bile ducts and those that may be classified as panbiliary. The major heritable bile duct disorders are those affecting the intrahepatic ducts, namely syndromic bile duct paucity, or Alagille syndrome, and the fibrocystic cholangiopathies autosomal recessive polycystic kidney disease/congenital hepatic fibrosis, and autosomal dominant polycystic kidney disease. This discussion focuses on heritable disorders of the bile ducts.