Type 1 diabetes is associated with a substantially increased risk of cardiovascular disease that might not always be appreciated in view of the fairly young age of patients with this condition. In fact, in type 1 diabetes, the heart is subject to a variety of pathological insults, including accelerated atherosclerosis, cardiac autonomic neuropathy, and possibly intrinsic cardiomyopathy. Although the relation between hyperglycaemia and microvascular complications has been well established, a direct effect of hyperglycaemia on cardiovascular disease in type 1 diabetes has long been debated. More recently, several studies, most notably the Diabetes Control and Complications Trial/Epidemiology of Diabetes Interventions and Complications, have clarified this issue and provided conclusive evidence that hyperglycaemia is indeed a mediator of cardiovascular risk in type 1 diabetes and that intensive diabetes therapy can reduce cardiovascular disease outcomes. We review current concepts in type 1 diabetes and the heart, focusing on recent insights into the central role of hyperglycaemia.