Introduction: The term hepatobiliary tuberculosis refers to the rare and localized form of hepatic tuberculosis as a distinct clinical entity, with signs and symptoms related to the hepatobiliary tract. We aimed to report a case of the primary biliary tuberculosis with subsequent pulmonary presentation. Case Presentation: A 28-year-old woman was admitted to our hospital due to recurrent abdominal pain, low-grade fever, and icterus and with a temperature of 38.2°C. According to magnetic resonance cholangiopancreatography (MRCP) with bile duct dilatation, IgG4, and high eosinophil with impression of autoimmune cholangiopathy, corticosteroid was administered. A few weeks later, she was hospitalized again because of fever and leukocytosis and the creation of multiple lymph nodes in the neck and abdomen. Biopsy of cervical lymph node was performed, which was not diagnostic. Smear, culture, and polymerase chain reaction (PCR) to detect tuberculosis were negative. According to eosinophilia and hypointense masses with the possibility of parasitic causes (Fasciola hepatica) triclabendazole was started. The symptoms briefly improved and she had little weight gain, but four months later she was hospitalized with pulmonary symptoms. Culture and sputum smear were positive for acid-fast bacilli. Conclusions: Hepatobiliary tuberculosis has delayed presentations with nonspecific manifestations. In our patient, despite that clinical presentations matched those of hepatobilliary tuberculosis, multiple biopsies from the abdominal and prepheral lymph nodes could not verify it. Therefore, diagnosis was made with delay, and finally, pulmonary presentation and positive smear for acid-fast bacilli confirmed tuberculosis.
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