PFIC3 (progressive familial intrahepatic cholestasis type 3) is an inherited cholestatic disorder caused by mutations in the ABCB4 gene encoding the Multidrug Resistance Protein 3 (MDR3) protein. 1 PFIC3 typically presents during infancy or early childhood, often progressing to chronic liver disease and cirrhosis, requiring liver transplantation. 2 Since the clinical features of PFIC3 overlap with many other forms of liver disease in childhood, definitive diagnosis may be problematic or delayed. Here, we report 2 patients, ultimately diagnosed with PFIC3, who initially presented with liver histological features including marked hepatic copper accretion that were considered indicative of Wilson disease (WD).