Laubry and Pezzi syndrome is a rare but serious complication of ventricular septal defect that increase the infectious endocarditis risk. Authors report a case of an 18 years old young girl presenting an enterococcus infectious endocarditis associated to Laubry and Pezzi syndrome. Initial course is not favourable requiring a surgical treatment in the acute stage. Bacterial endocarditis combined with Laubry and Pezzi syndrome have a poor prognosis needing observation and strict preventive precautions when a favoring factor is present.