Chronic liver disease is associated with many pulmonary complications. Several, including hepatopulmonary syndrome, portopulmonary hypertension, and hepatic hydrothorax have been extensively reviewed. However, hepatobiliary manifestations of primary pulmonary diseases have received less attention. This review focuses on hepatobiliary complications of respiratory failure, cystic fibrosis, α-1 antitrypsin deficiency, sarcoidosis, and tuberculosis.