The clinical diagnosis of double chamber right ventricle (DCRV) is not straightforward. Clinical history, clinical examination, 12-lead electrocardiogram, chest X-ray, and Echocardiography (echo) contribute to morphological diagnosis. Cardiac catheterization is essential for hemodynamic evaluation. A thorough presurgical workup helps the cardiac surgeon to choose the appropriate surgical approach and timing of surgery in an individual case. We present a case of a DCRV who presented to us in the fifth decade of life. Echo confirmed the morphological diagnosis and cardiac catheterization complemented the exact pull back gradient across the obstruction in the right ventricle. This patient was suggested muscle bundle resection and ventricular septal defect closure using right atrial approach.