Severe cholestatic hepatitis due to Coxiella burnetii is a rare form of clinical presentation of acute Q fever that is only occasionally detected in association with this infectious disease. We report a case of severe cholestatic hepatitis due to acute Q fever, with clinical signs of hepatic insufficiency, marked cholestasis, and renal insufficiency. The results of the initial serologic study using a complement fixation test were negative on two occasions, and transjugular biopsy and serological analysis with indirect immunofluorescence were required to establish the diagnosis. Despite treatment with doxycycline and ciprofloxacin, the cholestasis progressively worsened, with the development of hepatic and renal insufficiency, which rapidly disappeared after corticosteroid treatment.