Q fever is a zoonosis caused by a Coxiella burnetii. Q fever is clinically variable, presenting as asymptomatic infection, pneumonia, hepatitis and endocarditis. Treatment of acute Q fever with doxycycline is usually successful. Autoantibodies, such as anti-mitochondrial antibodies, smooth muscle antibodies (SMA), anti-cardiolipin and lupus anticoagulant, often rise in acute Q fever infection. Some cases may occasionally meet the criteria for autoimmune disease like systemic lupus erythematosus. We report a first case of Q fever that may mimic systemic lupus erythematosus in Korea.