Objective To explore how to differentiate the epilepsy and syncope in order to minimize the misdiagnosis. Methods Retrospectively analyzed the medical record of 6 cases which were misdiagnosed as epilepsy or syncope during April 2008 to September 2012 and reviewed the literatures about the differential diagnosis. Results Among the clinical characteristics, the ictal positional tone and loss of consciousness as well as the duration of postictal confusion are very important to the differential diagnosis. The ictal EEG shows highly rhythmic abnormal discharges when epileptic seizures occur. However, the ictal EEG would become slower and flatler during syncope. Conclusions When the automomic disorder and signs such as chest distress, arrhythmia. appear, the causes should not be limited in the cardiac diseases, the functional or structural abnormalities of the nervous system innervating the heart should also be considered; on the contrary, convulsions might not only due to the abnormal electrical activity in the brain, but syncope.