Left ventricular outflow tract (LVOT) obstruction with systolic anterior motion (SAM) of mitral valve is not only limited to patients with hypertrophic cardiomyopathy. A diagnosis of LVOT obstruction with SAM is important because conventional inotropic support may potentially aggravate hemodynamic deterioration. We present a case of LVOT obstruction with SAM in a patient who underwent an emergent surgery for ascending aortic dissection with pericardial effusion. The patient showed refractory hypotension after standard pharmacologic interventions during induction of anesthesia. Transesophageal echocardiography (TEE) revealed LVOT obstruction with SAM and it was managed appropriately under the guidance of TEE. Intraoperative TEE can play an important role in diagnosis and management of LVOT obstruction with SAM caused by pericardial effusion.