Coxiella burnetii, the agent of Q fever, produces a variety of clinical syndromes. The most frequent and serious chronic presentation is endocarditis, which presents unspecifically as a blood-culture negative endocarditis. It occurs almost exclusively in patients who have pre-existing valvular disease or who are immunocompromised. Without prompt recognition and appropriate antimicrobial therapy, the course of Q fever endocarditis is severe and potentially fatal. The epidemiology, signs and symptoms, laboratory findings, diagnosis and treatment of Q fever endocarditis are presented in our review.