Mutations within the BSEP and MDR3 gene can lead to different cholestatic diseases, e.g. progressive familiar intrahepatic cholestasis type 2 and 3 (PFIC2 and PFIC3) [20], [21], [22], benign recurrent intrahepatic cholestasis type 2 (BRIC2) [23] or intrahepatic cholestasis of pregnancy (ICP) [24] and low-phospholipid associated cholestasis (LPAC) [25]. The gene discussed is ABCB4; the disease is benign recurrent intrahepatic cholestasis type 2.