Introduction. SUDEP is not so rare event, unexplained, underused and underestimated. Its awareness has recently contributed to a number of initiatives for global action and clinical and experimental research. Aim. To undertake a literature review so as to update various aspects of SUDEP: pathophysiological mechanisms, potential markers for autonomic dysfunctions and risk factors, AEDs effects, non-AED management options, forensic/autopsy, patient-physician communications in patients with SUDEP risk. Method. A literature review, up to Nov. 2012, was conducted using PubMed-Medline for SUDEP, no indexed citation and relevant papers. Review. Interactions between the central and peripheral origin of cardiac and respiratory dysfunctions, triggered by epileptiform discharges in the cortical representation of the autonomic functions, may lead to SUDEP during simple partial autonomic seizures -even without other components of a seizure. A number of potential biomarkers of autonomic dysfunctions and risk of SUDEP are identified and proposed to use in its prevention: heart rate variability, long and short QT, arrhythmias, asystole, oxygen desaturation, apneas, hypoxia, postictal EEG suppression, circadian seizure pattern. Conclusions. Risk factors for SUDEP, AEDs effects, non-AED management of preventive options and forensic autopsy in diagnosis of SUDEP are discussed. Periictal long term video EEG, ECG and oxygen saturation monitoring may contribute to better understanding of SUDEP mechanisms and eventually to its prevention. SUDEP occurs as fatal coexistence of several predisposing risk factors. Diagnosis of SUDEP is underestimated and underused. In patient with high risk factors for SUDEP, in particular, with AED noncompliance, prognosis of epilepsy should be discussed with patient. Key words: SUDEP • animal seizure models and autonomic dysfunctions • potential biomarkers of SUDEP risk • risk factors of SUDEP • AEDs • autopsy atives were undertaken to assess the state of knowledge about SUDEP and to work out recommendations, e.g. multidisciplinary workshop to refine current lines of Copyright and photocopying by Foundation of Epileptology, 213