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Tetralogy of fallot (TOF) is one of the most common congenital heart disease (CHD) in children. With the development of pediatric surgery and intensive care units, increasing number of grown-up CHD patients are presenting for non-cardiac surgeries. Non-operated TOF patients suffer from chronic hypoxia and decreased pulmonary blood flow resulting in considerable alteration in the physiology. The optimal management of these patients, therefore, require a thorough understanding of the pathophysiology of the uncorrected TOF. We hereby report a case of successful management of a 10-year-old child with an uncorrected TOF posted for tibial external fixation device.