In this case report we present a 62-year-old patient with unrepaired tetralogy of Fallot who underwent a Potts shunt for palliation. Survival into late adulthood of patients with unoperated tetralogy of Fallot is rare. This patient is currently in New York Heart Association (NYHA) class II. A Potts shunt is associated with severe complications due to pulmonary vascular disease. To our knowledge, this is the first case report on a patient who survived into late adulthood with minor symptoms due to balanced hemodynamics after a Potts shunt.