Double-chambered right ventricle is an uncommon congenital heart disease, studied mostly by angiography, characterized by the division of the right ventricular cavity into two different pressure chambers. To analyze the anatomic features of this disease, data from 13 patients examined by echocardiography at the Mayo Clinic were reviewed. Despite the anatomic variety of this abnormality, two main types were identified. In the first type, intraventricular obstruction was due to an anomalous muscle bundle crossing the right ventricular cavity from the interventricular septum to the parietal wall. In the second type, no anomalous bundles were identified, and interventricular obstruction was due to marked parietal and septal hypertrophy. The main interventricular gradient was higher in the first type, and a ventricular septal defect was found to be associated more commonly with the second type.