The pulmonary tuberculosis is endemic in India, and the incidence of extensively drug-resistant tuberculosis (XDR-TB) is also rising. Though the incidence of pulmonary tuberculosis is high in acyanotic congenital heart disease, in Tetralogy of Fallot, which is a low flow condition, this association is infrequent. We report a rare association of extensively drug-resistant tuberculosis and Tetralogy of Fallot in a young adult patient who presented with breathlessness on exertion, hemoptysis and frequent respiratory infections. The patient was successfully managed by bronchial artery embolization, venesections, pulmonary outflow valvuloplasty and regime for Extensively drug-resistant tuberculosis.