We report on a 14-year-old girl with tetralogy of Fallot and the non-confluent main pulmonary artery with the left pulmonary artery after a left Blalock-Taussig shunt. She underwent a left-sided classic Blalock-Taussig shunt at the age of one. However, she showed repeated dyspnea and increasing cyanosis, and cardioangiography showed the non-confluent main pulmonary artery with the left pulmonary artery. Corrective surgery of tetralogy of Fallot was done by a two-staged operation. In the first stage, a EPTFE prosthesis was grafted between the main pulmonary artery and the left pulmonary artery, and in the second stage, intracardiac operation was performed. The late postoperative status and hemodynamic results were satisfactory and we emphasize the rational for this approach.