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Intracranial lipomas in an infratentorial and extra-axial location are extremely rare. The presented case of an extensive lipoma of the cerebellopontine angle (CPA) represents 0.05% of all CPA tumors operated on in our department from 1978 to 1996. The lipoma constitutes an important differential diagnosis because the clinical management differs significantly from other CPA lesions. The clinical presentation and management of the presented case are analyzed in comparison to all previously described cases of CPA lipomas. The etiology and the radiological features of CPA lipomas are reviewed and discussed. CPA lipomas are maldevelopmental lesions that may cause slowly progressive symptoms. Neuroradiology enables a reliable preoperative diagnosis. Attempts of complete lipoma resection usually result in severe neurological deficits. Therefore, we recommend a conservative approach in managing these patients. Limited surgery is indicated if the patient has an associated vascular compression syndrome or suffers from disabling vertigo.