A 31-year-old male of two-chambered right ventricle with ventricular septal defect, complicating infective endocarditis and tricuspid regurgitation, was presented. Two-dimensional echocardiographic study demonstrated tricuspid vegetations and a hypertrophied, anomalous muscle bundle in the right ventricle. Cardiac catheterization revealed 58 mmHg pressure gradient between inflow chamber and outflow chamber of the right ventricle. It seems that tricuspid regurgitation was resulted from infective endocarditis. He underwent resection of anomalous muscle bundle, repair of ventricular septal defect, and tricuspid valve replacement with satisfactory result. It has not been reported in Japan so far that tricuspid valve replacement was performed for the treatment of tricuspid regurgitation due to infective endocarditis in the patient with two-chambered right ventricle. In our case, cardiac catheterization was performed after subsidence of infective endocarditis. As echocardiography can detect vegetations and anomalous muscle bundle precisely, surgical intervention would be performed without cardiac catheterization in the case of infective endocarditis intractable to medical therapy.