Abstract The surgical treatment of Ebstein's anomaly is discussed from our experience with 5 patients who underwent successful operation together with 32 previously reported patients. Tricuspid valve replacement with a disc valve and primary closure of the atrial septal defect was performed in our 5 patients. In 3 patients plication of the atrialized ventricle with paradoxical movement was also employed. In the remaining 2 patients plication was not necessary. We believe that tricuspid valve replacement will usually be required for these patients. The low-profile Kay-Shiley disc valve with the Kay muscle guard is well suited for tricuspid valve replacement in patients with Ebstein's anomaly. The prosthesis should be placed above the coronary sinus to avoid injury to the conduction system. Elimination of the atrialized ventricle is an essential aspect of the procedure when paradoxical distention persists following repair or replacement of the tricuspid valve. Primary closure of the atrial septal defect also should be performed. All 5 of our patients were improved and remain in good condition four months, eleven months, one and one-half years, one and three-fourths years, and five years, respectively, following operation.