Since some PFIC gene variants in ABCB11, ABCB4, and TJP2 are described in patients with liver tumours, subjects with idiopathic chronic cholestasis and personal or familial risk factors for inherited cholestasis as well as DIC, ICP or LPAC history, should be screened for a panel of primary cholestasis-related genes and might likely benefit from monitoring with periodic ultrasound exams. The gene discussed is ABCB4; the disease is cholestasis.