Brucella endocarditis is a rare, and life threatening complication of human brucellosis. Children are usually infected by consumption of contaminated dairy product. Clinical presentation is similar to rheumatic fever. Aortic valve is most commonly involved. Clinical manifestations of fever, arthritis/arthralgia, aortic insufficiency, echocardiographic evidence of vegetation, and negative findings of blood culture, in a case with suspicious contact should suggest the diagnosis. Perioperative antibiotics combined with surgical treatment are an effective management. In pediatric age group, surgical techniques using biologic valves and preservation of native valves are preferred.Keywords: Aortic Valve Insufficiency; Brucellosis; Endocarditis; Therapeutics; General Surgery Implication for health policy/practice/research/medical education: Brucellosis is a common infection in Iran, and brucella endocarditis is a fatal cardiac involvement. Recognizing this life threatening complication is useful for pediatricians.