The worldwide epidemiology and population-based incidence of Q fever endocarditis (QFE) have been less well studied than those for uncomplicated Q fever. An exhaustive literature review revealed 408 patients with QFE reported between 1949 and 1994, mostly from 3 large geographic areas. Underlying valvular heart disease was almost invariably present, and 38% had prosthetic valves. The most common clinical manifestations were fever and congestive heart failure. The mortality rate dropped over the years from 65% to 25%, but a meta-analysis of published data showed the death rate to be significantly lower among patients receiving combination therapy (12/65, 18%), as compared to patients treated with tetracycline alone (18/41, 44%, p = 0.005). A 10-year (1983-1992) retrospective nationwide survey of QFE in Israel revealed 35 patients with QFE, representing an annual incidence of 0.75 cases per 1 million population. Underlying heart disease, clinical manifestations and outcome in the Israeli group were not substantially different from those described in the world literature. The current state-of-the-art clinical approach includes early diagnosis, prompt initiation of combination therapy for at least 3 years, and long-term clinical and serologic follow-up. Adherence to these rules might have contributed to the improved prognosis in recent years.