We describe the midterm result after Starnes operation for a severely symptomatic neonate with Ebstein's anomaly by reviewing the reported cases. A 4-day-old baby with "wall to wall heart" underwent Starnes operation. The tricuspid valve was closed without fenestration and the coronary sinus was drained into the right ventricle. Consequently, she underwent bidirectional Glenn shunt and Fontan operation at 6 and 23 months, respectively, and is now attending kindergarten without limitation. The follow-up study at 5 years revealed normally sized left ventricle with good function and the small right ventricle in the absence of thrombus formation with systolic forward flow.