Tetralogy of Fallot (TOF) is the most common etiology of congenital cyanotic heart disease, and Blalock-Taussig shunt (BTS) operation is considered the first-step management to maintain pulmonary blood flow in TOF patients. Complete repair of TOF is the standard surgical treatment that should be performed in infancy or early childhood for improved long-term survival. However, the prognosis of TOF patients treated by only palliative operation remains uncertain. We report a man with TOF underwent classic BTS operation at 2 years of age. Despite no medication, he had a long asymptomatic life. At 53 years of age, he started to complain of dizziness and recurrent attacks of syncope due to complete heart block (CHB) and inserted a permanent pacemaker. 2D and 3D Echocardiography showed uncorrected TOF with an overriding aorta, two ventricular septal defects (VSDs) were seen; a large inlet type VSD and another small muscular one with marked right ventricular hypertrophy with marked infundibular stenosis. Cardiac Computed Tomography (Cardiac CT) showed TOF with noted severely stenotic right ventricular outflow tract and pulmonary flow was maintained through a patent classical BTS between left subclavian artery (SCA) and the left pulmonary artery (LPA). Due to his stable condition, he was discharged on close up follow-up visits.