A male patient with single ventricle pulmonary stenosis, and persistent left superior vena cava underwent original Blalock-Taussig shunt (BTS) at 2 years of age and suffered from infective endocarditis at 38 years of age. A systemic work-up detected dural arteriovenous fistula and aneurysmal dilatation of the original BTS. Cardiac catheterization and cardiac magnetic resonance imaging revealed an appropriate pulmonary vasculature for bidirectional Glenn anastomosis and sufficient antegrade pulmonary blood flow through the pulmonary valve. Bilateral bidirectional Glenn anastomosis and resection of the aneurysm of the BTS-associated aneurysm were successfully performed.