Infectious endocarditis (IE) is a rare, but potentially fatal disease in pediatric patients. Most patients have underlying hemodynamic problems that predispose them to the risks of IE. Modified Duke criteria are commonly used for clinical diagnosis of IE. Though Streptococcus viridans and Staphylococcus aureus are the main pathogens of the disease, other pathogens have also been isolated. Herein we describe the first reported case of infective endocarditis caused by Pseudomonas oleovorans a girl with operated ventricular septal defect (VSD).