Myocarditis has only rarely been described as a manifestation of acute Q fever. Among our series of 1276 patients in whom acute Q fever was diagnosed during 1985--1999, myocarditis was diagnosed in 8. Two patients (25.0%) developed cardiac symptoms during the course of interstitial pneumonia, 2 (25.0%) initially presented with unexplained fever, and 1 (12.5%) presented with febrile cutaneous rash. In 3 patients, cardiac symptoms were inaugural: 1 patient experienced heart failure, and 2 experienced precordial pain. Dilated cardiomyopathy was documented in 7 patients, and 2 (1 of whom had undergone heart transplantation) died despite therapy. In addition, 1 patient was scheduled for heart transplantation because of cardiac insufficiency. When the patients in this study were compared with 32 control patients with acute Q fever, no specific epidemiological or clinical features were associated with this disease except worse prognosis (P=.006). Moreover, among the 12 patients from our series who died as a result of acute Q fever, 2 patients, who were significantly younger than the other 9 patients (P=.03), had myocarditis. Our study highlights the severity of Coxiella burnetii myocarditis.