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Q fever is nearly a worldwide zoonotic infection caused by the intracellular bacterium Coxiella burnetii, which is endemic in Israel. Acute infection mostly presents as a self-limited febrile illness, and occasionally involves respiratory or hepatic disease. Immune reactivation in the setting of acute illness was previously reported as well as various infectious positive cross-reactive serologic tests during the course of disease. Other autoimmune manifestation is infectious-type antiphospholipid syndrome linked to acute Q fever.Herein, we report a case of acute Q fever–related antiphospholipid syndrome with features resembling systemic lupus erythematosus treated successfully with corticosteroids. We review the literature and discuss the present state of knowledge on the link between Q fever and autoimmunity, aiming to raise clinicians' awareness of this possible rare presentation of the disease.