We report an extremely rare case of intracranial lipoma that was associated with subcutaneous lipoma situated on the anterior fontanelle. During ultrasonographic examination of the subcutaneous lesion, intracranial lipoma was incidentally diagnosed. Computed tomography revealed corpus callosum agenesis, interhemispheric lipoma extending into the choroid plexus. Magnetic resonance imaging demonstrated the subcutaneous lipoma and also the exact extent of the lesion. The pathogenesis of lipomas is discussed and the literature is reviewed.