A 73-year-old man was admitted with fever and abnormal liver function test. After admission, his fever persisted in spite of administrating a variety of antibiotics. He was diagnosed as acute Q fever on the basis of a high titer of IgM antibodies against phase II Coxiella burneti. His fever resolved after starting minocycline, but biliary enzymes continued to be elevated. Transbronchial lung biopsy and percutaneous liver biopsy revealed caseating granulomas and Mycobacterium tuberculosis was detected by culture. Miliary tuberculosis was diagnosed and his biliary enzymes declined after starting therapy with antituberculous drugs. This report of miliary tuberculosis complicating acute Q fever is a valuable and unprecedented case.