There are few reports of cone reconstruction for neonatal Epstein's anomaly. We report 14-day-old girl with symptomatic Ebstein's anomaly with prenatal diagnosis prenatally and who was born by caesarean section. Continuous infusion of prostaglandin was started immediately and she was transferred to our institution. The cardiothoracic ratio (CTR) was 74% on chest roentgenograph. The echocardiograph showed severe tricuspid valve regurgitation (TR) and functional pulmonary atresia. Despite aggressive medical therapy, congestive heart failure had advanced and early surgical intervention was required. The anterior leaflet was severely plastered to free the wall, like the Carpentier type C. After cone reconstruction, the intraoperative echocardiography showed mild to moderate TR, antegrade flow to the pulmonary arteries and good function of both ventricles. The patient was extubated on the 6th postoperative day and stayed in the intensive care unit for another 2 days. The CTR was 58%, and the echocardiography showed a well-functioning RV with mild to moderate TR and a right to left transatrial shunt flow with an arterial saturation level of over 90%. Cone reconstruction might even be effective in such border line cases of biventricular repair with dysplastic anterior leaflet of tricuspid valve.