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 hypertorphied aberrant muscle bundles. The aberrant muscle bundles traverse the right ventricle from the region of crista supraventricularis to the lower part of the anterior wall of the right ventricle. The“Two-chambered right ventricle”is usually associated with other congenital cardiac anomalies such as ventricular septal defect, pulmonary valvular stenosis, etc. Therefore this anomaly could be mistakenly diagnosed as Tetralogy of Fallot or isolated interventricular septal defect. The need to separate this entity from other types of infundibular stenosis is emphasized because of the important surgical implication. Authors recently experienced a case of“Two-chambered right ventricle”resulting from aberrant muscle bundles, that are associated with other cardiac anomal es i.e., pulmonary valvular stenosis, a/splastic tricuspid valve with regrugitation and partial anomalous pulmonary venous return to the right atrium. Here were present the findings of E.K.G., cardiac catheterization, simple chest PA, cine-right ventriculography, and autopsy together with a review of related articles.