Progressive familial intrahepatic cholestasis type 2 (PFIC2) is a rapidly developing hepatic disease that leads to
early childhood cirrhosis and liver failure. We present a diagnostically challenging case of a 20-year-old male with 8 years
history of recurrent icteric episodes and constantly normal serum levels of GGT. Genetic study disclosed two novel mutations
in gene ABCB11 and liver histopathology provided evidence of intrahepatic cholestasis with slowly progressing fibrosis.
Concurrent diseases were cholelithiasis and chronic calcifying pancreatitis.