'Cardiomyopathy' (CM) is defined by the World Health Organization as 'a disease of the myocardium associated with cardiac dysfunction'. In a child, it is associated with a significant risk for anaesthesia. In addition, cardiac arrest under anaesthesia has been attributed to an undiagnosed CM. Care of these patients is complicated by the fact that there are several different forms of CM that have differing anaesthesia management goals, aimed at maintaining the patient's baseline haemodynamic variables of preload, heart rate, contractility, and afterload. With the emergence of new diagnostic tools, together with advances in cardiac imaging and improved treatment modalities (such as ventricular assist devices), the anaesthetic management of a child with a CM is evolving. This review describes the different forms of the disease in terms of pathology, aetiology, and clinical presentation. Dilated, hypertrophic, and restrictive CM are the most common forms. We examine recent advances in therapy, including the management of severe end-stage disease, while highlighting the specific anaesthetic considerations for children with each type of CM.