The defective CFTR channel in the bile ducts adds to the pathogenesis of CF-related liver disease (CFLD), initially featuring a toxic cholestatic pattern [9] and progressing to focal or multifocal biliary cirrhosis and end-stage liver cirrhosis that contributes to the appearance of other complications such as portal hypertension, ascites, esophageal varices, and in some cases to liver failure [20]. Here, CFTR is linked to cystic fibrosis-related liver disease.