Cyanotic neonates with Ebstein's anomaly associated with pulmonary atresia are a subgroup with the high mortality rate in pediatric cardiac surgery. We report a case of a severely symptomatic low birth weight neonate with Ebstein's anomaly and pulmonary atresia. A biventricular repair with Danielson-type tricuspid valve repair was performed. The baby tolerated the procedure with adequate hemodynamics, but died of peritoneal hemorrhage on the second postoperative day. This report could be an addition to the recent literature regarding the feasibility of biventricular repair for symptomatic neonates with Ebstein's anomaly even in low birth weight neonates.