Tuberculosis is known to involve the liver in different ways. The term hepatobiliary tuberculosis refers to the localized form of hepatic tuberculosis as a distinct clinical entity, with signs and symptoms related to the hepatobiliary tract. Its clinical features and the different diagnostic aids used in its diagnosis are reviewed. Plain abdominal radiographs showing diffuse hepatic calcifications seen in approximately 50% of cases are almost diagnostic for hepatobiliary tuberculosis. Liver biopsies obtained either by ultrasound, computed tomography or laparoscopy, showing caseating granuloma usually establish the diagnosis. In the absence of caseation necrosis, a positive acid-fast bacillus (AFB) or culture for Mycobacterium tuberculosis is needed to establish the diagnosis. A polymerase chain reaction assay for the identification of Mycobacterium tuberculosis in liver biopsy specimens is a new development. Treatment is similar to that used for pulmonary tuberculosis. Quadruple therapy (using four anti-tuberculosis drugs) is recommended, generally for 1 year. For patients with obstructive jaundice, in addition to anti-tuberculous treatment, biliary decompression should be performed either by stent insertion during endoscopic retrograde cholangiopancreatology, by percutaneous transhepatic biliary drainage or by surgical decompression whenever feasible.