The cardiac complications of certain neurologic diseases have been well recognized for over 50 years and are mostly evident for cerebrovascular accidents. Although these complications are frequent and in most circumstances benign, detrimental cardiac side effects, such as serious arrhythmias and myocardial infarctions, may occur. The link to most of these cardiac derangements is a transient or chronic autonomic dysfunction, depending on the specific neurologic disease. Myocardial infarcts, left ventricular dysfunction, and arrhythmias are well-recognized complications of subarachnoid hemorrhage, intracranial bleed, and ischemic strokes. Seizures may present with atonia or sudden death from asystole. Degenerative brain disorders, namely the synucleinopathies, may affect the central control areas or peripheral ganglia of the autonomic nervous system, causing autonomic dysfunction. In addition, cardiac conduction defects and cardiomyopathy are common in certain neuromuscular disorders, namely the dystrophies and mitochondrial myopathies.