Hypertrophic cardiomyopathy (HCM) poses many unique challenges regarding the conduct of anesthesia and surgery. Adequate preload, control of sympathetic stimulation, heart rate, and increased afterload are required to decrease the left ventricular outflow tract obstruction. Comprehensive intraoperative transesophageal echocardiography (TEE) examination confirms the diagnosis, elucidates the pathophysiology, and identifies the various anomalies of mitral valve apparatus and allows assessment of the adequacy of surgery. In this review, we focus on the preoperative assessment, conduct of anesthesia and comprehensive TEE examination of patients presenting for surgery with HCM. The various surgical options are extended myectomy and resection, plication and release.