Arrhythmogenic right ventricular dysplasia/cardiomyopathy is a genetic disorder characterized by fibrofattty replacement of the right ventricular myocardium. In the revised 2010 Task Force Criteria, a major criteria for ARVD/C is the presence of RV aneurysm by 2D echo. Our report demonstrates that intracardiac ultrasound can detect RV aneurysms and also focal absence of trabeculations which brings additional value to the diagnosis of ARVD/C. A 26-year-old male patient suffering from multiple sustained episodes of ventricular tachycardia was implanted with an internal cardiac defibrillator after confirmation of the disease by cardiac magnetic resonance imaging. Intracardiac ultrasound was performed using a 6F, 9 MHz catheter and the iLAB intravascular system (Boston Scientific). Images of right ventricular inflow tract, outflow tract, apex and pulmonary artery were taken and saved on videotape. ICE revealed loss of trabecular structure at the right ventricular outflow tract with an antero-septal aneurysm at this level. Intravascular ultrasound provides useful information on the presence of aneurysms and of tissue characterization.