Intracranial lipomas (ICLs) are rare congenital malformations associated with some central nervous system anomalies and clinical findings. The aims of this study were to assess the locations, associated malformations, and imaging and clinical findings of patients with ICLs. Based on published reports, the neuroradiologic features, clinical symptoms and signs, differential diagnosis, and surgical indications are discussed. Seventeen cases of ICL were reviewed, on which the results of neurologic and radiologic examinations were based, including computed tomography (CT) and magnetic resonance imaging (MRI) findings. A total of 20 lipomas were observed in 17 patients. The locations of the lipomas were interhemispheric fissure and/or callosal (45%), choroid plexus and/or intraventricular (15%), pericallosal (10%), the quadrigeminal plate and/or superior cerebellar cistern (10%), the prepontine cistern (10%), hypothalamic and/or suprasellar (5%), and the Sylvian fissure (5%), respectively. Associated midline anomalies were observed in 3 patients with callosal and/or pericallosal lipoma and in I patient with a Sylvian fissure lipoma. Two patients had epilepsia. One patient had a lipoma of the quadrigeminal plate cistern causing obstructive hydrocephalus by compression and partial obstruction of the aqueduct. Presenting complaints and results of the neurologic examinations were not associated with lipomas in the other 14 patients. CT and MRI easily showed the fatty content of the ICLs and associated anomalies in all patients. ICLs are mostly located midline and discovered incidentally. Sometimes, there are associated midline anomalies and vascular variants; the most common associated neurologic symptom is epilepsy They rarely require surgical intervention.