Background and Aim: A sinus of Valsalva aneurysm (SVA) is a rare cardiac anomaly. Rupture of a SVA often causes hemodynamic instability due to intracardiac shunting or cardiac tamponade, therefore immediate diagnosis and urgent treatment are required. Methods: We report an 18-year-old female with cardiac tamponade due to rupture of a localized aneurysm of the right coronary sinus of Valsalva. No other congenital or acquired cardiac anomalies were found. Neurological observation precluded urgent surgery with heparinization and extracorporeal circulation. Results: Semi-urgently the SVA was successfully resected. Conclusions: Semi-urgent surgery for a ruptured aneurysm of the Sinus of Valsalva was successful. In selected cases off pump surgery can be contemplated.