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Tetralogy of Fallot (TOF) presenting with superior vena cava (SVC) syndrome is an exceedingly rare scenario. The diagnosis of this condition is still a difficult task due to its unspecific clinical features and unclear recommendations for the best diagnostic tools. We report an interesting case of a 23-year-old male who was found to have TOF with the presentation of SVC syndrome after a sequential diagnosis. The timely diagnosis helped in the successful management of the patient. Through this case, we want to make clinicians aware of such a rare association and its diagnosis.