Tetralogy of Fallot’s with pulmonary atresia is a duct-dependent cyanotic heart lesion. This is a combination of a ventricular septal defect, an overriding aorta, pulmonary atresia and right ventricular hypertrophy. There is a R→L shunt at ventricular level and a L→R shunt at ductal level to provide pulmonary blood flow. Symptoms depend on pulmonary arteries and additional collateral blood vessels from the aorta to the pulmonary circulation. Anaesthetic management can be challenging and needs to avoid decreasing pulmonary vascular resistance and avoid increasing systemic blood pressure.