Introduction: Little is known regarding the association between congenital hepatic fibrosis (cHF) and cholangiocarcinoma and publications reporting these associations are rare. In literature, only four cases reporting the associations exist; those four cases were all diagnosed at autopsy. Herein, we describe a case of cHF and intrahepatic cholangiocarcinoma that was successfully treated with surgical resection. case report: A 46-year-old male with past medical history significant for cHF for 12 years, complicated by portal hypertension and esophageal varices, was found to have lesions on MrI suspicious for intrahepatic cholangiocarcinoma. the patient had relatively preserved liver function, and the decision was made to proceed with surgical resection. Pathology showed poorly differentiated cholangiocarcinoma with background features of cHF. conclusion: All patients with cHF patients should be monitored