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. This evidence concerns the gene ABCB11 and benign recurrent intrahepatic cholestasis.