Among the bacteria of the HACEK group, Actinobacillus actinomycetemcomitans is the organism involved most commonly in infective endocarditis. However, the epidemiological and clinical features specifically associated with this species have not been evaluated adequately. Three patients with infective endocarditis caused by A. actinomycetemcomitans seen at the Hospital La Timone (Marseille, France) between 1994 and 2001 are reported. Of 99 cases in the literature, 75% of patients had previous heart disease before infective endocarditis, the portal of entry of which was usually the oral cavity. Among the total of 102 cases, 27 had prosthetic valves. Intermittent fever was observed in all cases, and weight loss and peripheral signs of endocarditis were noteworthy in this study. Anaemia and microscopic haematuria were frequently noted. The disease is insidious, with a mean duration of symptoms of 13 weeks before diagnosis, as confirmed by blood cultures incubated for > 5 days. The aortic valve is most commonly involved, and echocardiographic findings were non-specific. Complications occurred in 63% of patients, with emboli being the most common. The surgery rate was 23.5%. The overall mortality rate was 18%. Of the cases, 76.5% were cured with antibiotics alone, including a simple third-generation cephalosporin or a combination of ampicillin and an aminoglycoside. An antibiotic therapy duration of at least 4 weeks is recommended. Surgical therapy is usually required for haemodynamic reasons. Prophylaxis of A. actinomycetemcomitans endocarditis relies on antibiotic therapy for all cardiac patients at risk before dental procedures. Among 17 patients undergoing dental manipulations, only eight received amoxycillin before the procedure, demonstrating that prophylaxis is far from being systematically prescribed. In conclusion, A. actinomycetemcomitans endocarditis should be highly suspected in patients with previous cardiac disease and for whom symptoms have evolved over a number of weeks or even months.