The long term results of Tetralogy of Fallot (TOF) repair have been good, pulmonary regurgitation and consequent right ventricular dilatation is the most common long term sequel of definitive surgery. Even in such a setting, overt systemic venous congestion is limited to the rare setting where right ventricular dysfunction sets in due to deferred surgery or progressive arrhythmia. Here we report a unique case where a patient developed right heart failure from an unexpected etiology 14 years after TOF repair.