We report a 27-year-old woman who presented with worsening exertional. dyspnea and palpitation. Transthoracic echocardiography showed ventricular septal defect, with left-to-right shunting and mild aortic regurgitation detected. Cardiac catheterization was performed that revealed sinus of Valsalva aneurysm with rupture into the right ventricle. Transesophageal echocardiography (TEE) confirmed a rupture of sinus of Valsalva aneurysm and ventricular septal defect. The patient underwent elective surgical repair of the ruptured sinus and intraoperative TEE demonstrated severe aortic regurgitation after repair and mechanical prosthetic valve was implanted. This case illustrates that TEE frequently establishes the diagnosis and provides more information concerning additional cardiac lesions in a patient with ruptured sinus of Valsalva aneurysm. Aortic regurgitation should be evaluated after repair of rupture of sinus of Valsalva by TEE.