Two patients with Sylvian fissure lipoma are reported. One of them was operate on because of an increased frequency of epileptic seizures in spite of high dose carbamazepine treatment. A review of the literature regarding the surgical experience with Sylvian lipomas revealed only four cases, and in none of them was the lipoma removed totally. One of ours is the first case in which total removal of the lipoma was achieved. The postoperative course was uneventful and carbamazepine dose was gradually lowered without further epileptic activity. If these exceedingly rare lesions cause symptoms then surgical intervention is indicated. With microsurgical techniques, Sylvian fissure lipomas can be removed totally without complication and symptomatic improvement may result.