Pathogenic variants in the adenosine triphosphatase phospholipid transporting 8B1 (ATP8B1) gene cause progressive familial intrahepatic cholestasis type 1 (PFIC1) and benign recurrent intrahepatic cholestasis type 1 (BRIC1) (1). The gene discussed is ATP8B1; the disease is progressive familial intrahepatic cholestasis type 1.