Congenital hepatic fibrosis is a fairly uncommon disorder and it is usually associated with other fibropolycystic disorders, including renal involvement. Main clinical features are hepatic fibrosis, portal hypertension, and renal cystic disease. There are multifocal saccular dilatations of segmental bile ducts, usually with accompanying calculi formation and recurrent bacterial cholangitis. Renal cystic disorders are common complications of Caroli disease (CD), but renal amyloidosis is fairly uncommon. We present a patient who was diagnosed with CD at the age of 31 years and in whom renal AA amyloidosis developed at the age of 45 years due to recurrent bacterial cholangitis.