Schizophrenia is a devastating mental disorder, which is often associated with severe loss of functioning and shortened life expectancy. Suicides and accidents are well-known causes of the excess mortality, but patients with schizophrenia have also been reported to be three times as likely to experience sudden unexpected death as individuals from the general population. This review is aimed to offer an update of the prevalence and mechanisms for sudden cardiac death in schizophrenia. The PubMed database was searched from 1966 up to May 2007 with key words schizophrenia AND “ sudden cardiac death” OR “autonomic dysfunction” OR “torsades de pointes”. Part of the high death rates may be explained by long-lasting negative health habits, disease- and treatment-related metabolic disorders, and consequent increased frequencies of cardiovascular diseases. The antipsychotic medications may also increase the risk as some antipsychotics may cause prolongation of QT-time, serious ventricular arrhythmias and predispose to sudden death. Autonomic dysfunction seen as low heart rate variability and decreased baroreflex sensitivity may also contribute via malignant arrhythmias. Due to the complex interaction of various risk factors for sudden death, the patients need a comprehensive follow-up of their physical health. In addition, more studies on the role and prevalence of autonomic dysfunction in psychotic patients are needed.