A 2-year-old girl had a focal seizure two weeks before admission. A pre-operative computed tomography scan of the head revealed an enhancing tumor in the right temporal area. She underwent craniotomy and tumor removal later. On microscopic examination, the tumor was composed of sheets of neurons in a glial background. The diagnosis was ganglioglioma. The child was in good condition when last seen two years after the operation, without evidence of tumor recurrence on a subsequent image study. The pertinent literature regarding ganglioglioma is reviewed.