CFTR and benign recurrent intrahepatic cholestasis type 2: We report a 27-year-old woman presenting with benign recurrent intrahepatic cholestasis type 2 due to heterozygosity in <i>ABCB11.</i> Interestingly, she was also found to be heterozygous in cystic fibrosis transmembrane conductance regulator, <i>NPHP4</i>, and <i>A1ATD</i> (<i>SERPINA1</i>), which may explain the severe nature of her disease expression because heterozygosity in each of these genes has been associated with cholestasis.