Atypical presentations of tuberculosis might cause difficulties in diagnosis, especially in developing countries. Primary hepatobiliary tuberculosis is a rare condition and the diagnosis of this condition necessitates a high index of suspicion. In this report, we present a case with obstructive jaundice, dilated intrahepatic bile ducts and lymphadenopathies in the porta hepatis. Endoscopic retrograde cholangiopancreaticography showed irregularities, strictures and dilatations both in the intra- and extrahepatic bile ducts resembling sclerosing cholangitis. The liver biopsy showed caseous granulomatous hepatitis and Langhans giant cells compatible with tuberculosis, and a lymph node biopsy obtained from the left cervical region demonstrated caseating confluent granulomas with abundant acid-fast bacilli. The present case shows that primary hepatobiliary tuberculosis may mimic primary sclerosing cholangitis and should be considered in the differential diagnosis of patients with sclerosing cholangitis-like changes on endoscopic retrograde cholangiopancreaticography.