Heart rate variability (HRV) provides indirect insight into autonomic nervous system tone, and has a well-established role as a marker of cardiovascular risk. Recent decades brought an increasing interest in HRV assessment as a diagnostic tool in detection of autonomic impairment, and prediction of prognosis in several neurological disorders. Both bedside analysis of simple markers of HRV, as well as more sophisticated HRV analyses including time, frequency domain and nonlinear analysis have been proven to detect early autonomic involvement in several neurological disorders. Furthermore, altered HRV parameters were shown to be related with cardiovascular risk, including sudden cardiac risk, in patients with neurological diseases. This chapter aims to review clinical and prognostic application of HRV analysis in diabetes, stroke, multiple sclerosis, muscular dystrophies, Parkinson\'s disease and epilepsy.