ABSTRACT A 2-year-old child was diagnosed with subaortic ventricular septal defect (VSD) with severe infundibular and valvular pulmonary stenosis (PS) by transthoracic echocardiography. Intraoperative transesophageal echocardiography (TEE) detected aneurysmal interventricular and interatrial septum (IAS), subaortic VSD, and right ventricular outflow tract (RVOT) with an additional midmuscular VSD. The aneurysmal interventricular septum (IVS) was repaired. An accessory tricuspid papillary muscle was attached to RVOT, which was augmented with a transannular pericardial patch to have minimal postoperative gradient without sacrificing the accessory papillary muscle (APM). This rare case poses a challenge to the anesthetist and surgeon in the form of diagnostic differences, severe right ventricle (RV) dysfunction with aneurysmal IVS, and difficulty in reconstruction of RVOT. Intraoperative TEE played a greater role to diagnose the IVS aneurysm, additional VSD and guide for appropriate surgery. How to cite this article Das S, Kumar A. Intraoperative Echocardiographic Detection of Septal Aneurysm and Additional Ventricular Septal Defect in a Child with Tetralogy of Fallot. J Perioper Echocardiogr 2017;5(1):21-24.