A patient with a ganglioglioma in the basal ganglia is presented. An 18-year-old man presented with involuntary movement in his right hand and right homonymous hemianopsia. Computed tomography and magnetic resonance imaging revealed a cystic tumor with an enhancing nodule in the basal ganglia extending into the thalamus. The mass was totally resected by a trans-distal Sylvian approach and was histologically proven to be a ganglioglioma. This is a rare case of midline ganglioglioma in a patient who underwent gross total resection.