Prematurity and Low birth weight have been an obstacle to early total correction of tetralogy of Fallot (TOF). However, the practice of waiting to obtain an optimal weight before surgery is risky and has not achieved desirable results. Stenting of the right ventricular outflow track (RVOT) has been reported as an optional palliative procedure to delay complete repair. We report a case of successful RVOT stenting in a low birth weight infant with TOF who had prostaglandin E1 dependency. A twin newborn was born by normal delivery at 36+5 weeks gestation, weighing 2150 grams. He was diagnosed as TOF with severely restricted pulmonary blood flow through echocardiography. At 7 days after birth, a 5mm diameter stent with 15mm length (Palmaz Genesis���, Cordis Corporation, FL, US) were inserted to RVOT in catheterization labortory room. But it was placed more distally than target position, so proximal portion of the infundibulum was not covered by it. So we additionally insert a 4mm/15mm stent. Six days after stent insertion, he could be go home with aspirin. The patient was well, except occasional transient cyanotic spell. At 76 days after stent insertion, lasting cyanosis developed. Echocardiography showed the stenosis of right ventricular outflow tract proximal to the stent. At that time, his body weight was 4.9 Kg. Corrective operation was performed and he was discharged to home 25 days after surgery. RVOT stenting might be a convincing optional procedure for waiting to get an optimal body weight in an low birth weight early neonate with TOF and prostaglandin E1 dependency. And careful attention is needed in positioning of stent which should widen the entire stenotic RVOT.