Cholangitis complicating congenital hepatic fibrosis.

A patient with cholangitis complicating congenital hepatic fibrosis is presented. In contrast to most other reported cases, many of whom have been treated surgically, this patient had a benign course. The likelihood that this complication of congenital hepatic fibrosis will respond to appropriate conservative therapy suggests that more aggressive forms of treatment should be reserved for those patients who fail to respond to medical management.