Ebstein's anomaly in combination with tetralogy of Fallot (TOF) is rare. We report a male infant with these combined anomalies who presented with severe cyanosis, heart failure, and respiratory distress after birth. Biventricular repair was successfully performed with 1-stage correction of his ventricular septal defect (VSD), right ventricular outflow tract (RVOT) obstruction, and tricuspid regurgitation. The downward displaced tricuspid valve was also restored to the normal annulus position. The infant recovered well. The surgical technique for this rare and severe anomaly is reported here.