Ganglioglioma in the temporal lobe was encountered in 0.6% of a series of 998 patients undergoing biopsy for intracranial tumors. It was more frequent in young adults and children. Seizures were the main symptom and had been present over a long period of time in some patients. Clinical examination also revealed intracranial pressure, focal signs, and behavioral problems, but these symptoms were not always evident. Electroencephalography was not specific. Calcifications were visible on computerized tomography (CT) in five cases. The cystic and well-circumscribed aspects of these tumors were apparent on CT scans; these features were confirmed at operation and on magnetic resonance imaging (MRI), when it was also found that the tumors were partially solid. Therefore, MRI seems to be a useful diagnostic method, particularly when a cystic tumor is revealed on CT, and is also valuable for postoperative monitoring. Surgical treatment for ganglioglioma is recommended, with total extirpation if possible.