Cardiomyopathy is a disease of the myocardium associated with cardiac dysfunction that cannot be explained by abnormal loading conditions or congenital heart disease. Dilated cardiomyopathy (DCM) is a phenotypic class of cardiomyopathy that is defined by ventricular chamber dilation with dysfunction that is secondary to ineffective systolic shortening. In children without known structural heart abnormalities DCM is the most common cause of congestive heart failure. The outcome of patients presenting with DCM is variable, with some children who present with fulminant heart failure requiring mechanical circulatory support followed by transplantation while others recover normal function. This chapter details the perioperative considerations involved in care of a child with severe DCM.