We report long-term outcome after two-stage, “one lung repair” in a four-year-old boy with tetralogy of Fallot and congenital absence of the left pulmonary artery. The operation was carried out two years after a palliative aortopulmonary central shunt and was uneventful. Twenty-six years later, the patient is in excellent clinical condition, with normal peripheral oxygen saturation. A recent radionuclide lung scan and cardiac magnetic resonance imaging show the pulmonary flow entirely directed into the right lung. In selected cases, the long-term prognosis of patients with tetralogy of Fallot and true absence of left pulmonary artery after “one lung repair” may be excellent.