CHF largely presents in childhood, manifested as portal hypertension and/or cholangitis, and is often associated with renal malformations, such as AR-PKD, renal tubular ectasia, or other forms of cystic kidney disease.[8] However, recent identification of the PKHD1 gene, implicated in AR-PKD, has confirmed that some cases of CHF are indeed due to PKHD1 mutations, found in 32.1% of adults with CHF or Caroli disease.[9] As we all know, CHF is a hereditary disease. Here, PKHD1 is linked to cholangitis.