A 50-year-old woman, diagnosed with severe rheumatic mitral stenosis, was referred for transesophageal echocardiography (TEE) study. Transesophageal echocardiogram was done to confirm suitability for percutaneous balloon valvotomy and to look for any evidence of thrombus in left atrial appendage (LAA). TEE evaluation followed by CT scan and MRI modality confirmed the patient having congenital absence of LAA – a rare occurrence with less than 25 reports in literature.