The "Two-chambered right ventricle" is a rare, but unique congenital cardiac anomaly characterized by subdivision of the right ventricle into proximal high pressure chamber and distal low pressure chamber by hypertrophied aberrant muscle bundles. The aberrant muscle bundles traverse the right ventricle from the region ofcrista supraventricularis to the lower part of the anterior wall of the right ventricle. The "Two-chambered right ventricle" is usually associated with other congenital caradiac anomalies such as ventricular septal defect, pulmonary valvular stenosis, etc. Therefore this anomaly could be mistakenly diagnosed as Tetralogy of Fallot orisolated interventricular septal defect. The need to separate this entity from other types of infundibularstenosis is emphasized because of the important surgical implications. Authors recently experienced a case of the "Two-chambered right ventricle" resulting from aberrant muscle bundles, that are associated with other cardiacanomalies i.e., pulmonary valvular stenosis, splastic tricuspid valve with regurgitation and partial anomalouspulmonary venous return to the right atrium. Here we present the findings of E.K.G., cardiac catheterization, simple chest PA, cine-right ventriculography, and autopsy together with a review of related articles.