Double-chamber right ventricle (DCRV) is a rare congenital heart disease consisting in right ventricular obstruction due to one or several anomalous muscle bundles that divide the right ventricle into two chambers. The right ventricular outflow tract obstruction is generally progressive in these patients. A ventricular septal defect is one of the commonly associated malformations. A 23-year-old woman with exertional dyspnea was admitted to our hospital and undertaken echocardiography, cardiac catheterization and both ventricular angiograms. The diagnosis was established and report with review of literatures.