A 62-year-old women had a history of cyanosis and easy fatigability from school age but had refused medical examinations. She was admitted to our department with exacerbation of dyspnea at the age of 59. Laboratory tests showed hypoxia complicated with polycythemia with 35 mmHg of PaO2, 8.5 x 10(6) erythrocytes per mm3. The systolic pressure of the right ventricle was 155 mmHg. RI angio demonstrated a high degree of the right-to-left shunt at the ventricular level. The patient was given oxygen therapy at home without diagnosis of tetralogy of Fallot (TOF) and eventually died at the age of 62. Tetralogy of Fallot was diagnosed at autopsy. Pulmonary stenosis of this case was located in infundibulum. The lining of the infundibular inlet was constituted of the thick fibrous tissue which remarkably augmented the pulmonic stenosis. Although the autopsy findings were considered to be relatively mild TOF, the death was presumed to be caused by development of the infundibular stenosis due to formation of fibrous tissue. This is the oldest patient in Japanese medical literature to be identified by cardiac catheterization and autopsy finding as a case of TOF.