Hypertrophic obstructive cardiomyopathy (HOCM) is a complex cardiovascular disorder affecting patients with varying degrees of cardiac manifestations. The optimal treatment strategy for patients with HOCM and end-stage liver disease (ESLD) undergoing evaluation for orthotopic liver transplantation (OLT) is not well defined. Although medical management is the accepted first-line treatment, symptomatic patients with severe left ventricular outflow tract (LVOT) obstruction unresponsive to medications may require further interventions prior to surgery. Perioperative cardiovascular adverse effects in HOCM patients during non-cardiac surgery are relatively high. The anesthetic management of HOCM patients can be challenging and involves understanding the many factors that can be expected to aggravate the dynamic LVOT obstruction during OLT. © 2011 Nova Science Publishers, Inc. All rights reserved.