Refractory gelastic seizure is one of the most common clinical manifestations in patients with hypothalamic hamartoma (HH) and HH is usually regarded as the epileptogenic focus. A young female patient with a small HH and refractory seizures is reported here. However,both the seizure semiology and results of electroencephalogram monitoring indicated the right temporal region was the epileptogenic focus. Thus a standard right anterior temporal lobectomy was performed while the hamartoma preserved. There was a marked improvement in both seizure frequency and quality of life during a 13-month follow-up. The outcome supported the concept that independent epileptogenic focus outside of the hypothalamus might occur in patients with HH.