In April 2004, Rammeloo et al. reported a child-aortic-valve endocarditis following pneumococcal meningitis. Classically, the association of pneumococcal pneumonia along with meningitis and infectious endocarditis (IE) is known as Austrian syndrome. We assessed the incidence, clinical manifestations and follow-up of patients diagnosed with Austrian syndrome in Lugo (Northwest Spain) between 1987 and 2002. A computerized MEDLINE search was also performed for literature review. During the study period 165 Lugo patients met criteria for definite IE. Two of the 3 patients with definite streptococcus pneumoniae IE also met definitions for Austrian syndrome. The incidence of this syndrome in Lugo patients with definite IE was 1.2%. In the literature review 48 cases, including our two patients, were found. Complete clinical information was only available on 16 patients. Alcoholism constituted the most common predisposing factor. Aortic valve involvement and high grade of valve regurgitation leading to cardiac failure and the need of cardiac surgery during the admission were common in these patients. This review confirms that Austrian syndrome is a rare but severe disease. Early recognition of these patients is required to avoid ominous complications.