Encephalitis is characterized clinically by fever, seizure, and an altered level of consciousness. Mycoplasma pneumoniae, a common respiratory pathogen, has been implicated as an etiology of encephalitis. The present study was designed to analyze status epilepticus associated with M. pneumoniae encephalitis in a series of children through retrospective review of cases between January 2002 and January 2008. Systematic clinical data were evaluated. Nine patients were identified: five girls and four boys, aged 4 years to 10 years. All were positive for M. pneumoniae by serology. Six of the nine children (67%) developed refractory status epilepticus. The major clinical symptoms included fever (100%) and upper respiratory symptoms (78%). The most common seizure type was primary focal with secondary generalized seizure (44%). The time of follow-up for this study ranged from 18 months to 86 months. At the end of the study period, two patients had died, seven had developed epilepsy or neurologic deficits, and none had returned to baseline. These data indicate that children with status epilepticus associated with M. pneumoniae encephalitis have high mortality and morbidity. Clinicians should be aware of the potential role of M. pneumoniae in status epilepticus.