An exceptional case of lipoma of the sylvian region is described and the other seven reported cases are reviewed. The sylvian fissure is the most rare site of intracranial lipomas. Sylvian lipomas may be asymptomatic or present with epileptic seizures due to irritation of the cortex of the sylvian fissure. Only two previous cases have been diagnosed during life by computerized tomography and operated on; the deep location of these lipomas and their adherences to the sylvian cortex and the branches of the middle cerebral artery make radical removal impossible and dangerous.