Epilepsy and seizures can have a dramatic effect on the autonomic nervous system by involvement of the central autonomic control centers. The peri-ictal changes can lead to short-term alteration of cardiac functions in patients with seizures, and are partially hemispheric specific. Changes in heart rhythm, conduction and even subtle signs of ischemia have been reported. Ictal asystole and the lock-step phenomenon during seizures play an important role in the pathophysiology of SUDEP. In patients with longlasting epilepsy and multiple seizures, there are now convincing arguments for a chronic dysfunction of the autonomic nervous system. In this sense, heart rate variability can be considered as a biomarker of autonomic dysfunction in epilepsy. Early recognition of these short- and long-term cardiac effects will become useful in predicting seizures and in guiding more individualized treatment in the near future.