With improvements in the surgical technique for orthotopic liver transplantation, patients with significant underlying systemic disease are considered candidates for transplantation, thus increasing the complexity of the medical management of these patients and necessitating additional monitoring in order to minimize the anesthetic risk. We describe the anesthetic management of orthotopic liver transplantation for a patient with severe hypertrophic cardiomyopathy and mitral insufficiency. In this case, transesophageal echocardiography proved useful in the management of the postreperfusion period of the surgical procedure.