A cerebellopontine angle (CPA) lipoma in a 17 year-old woman is reported. The patient was admitted for evaluation of episodic vertigo, preauricolar pain and progressive hearing loss on the right ear. Auditory brain stem evoked potentials revealed an interwave latency increase on the right ear. Computerized Tomography and Magnetic Resonance Imaging showed a low density lesion and high intensity signal, respectively, in the right CPA. Reports of surgically treated lipomas of the CPA are extremely rare. Our patient underwent a right lateral suboccipital craniotomy and a subtotal resection of the lesion. Histological examination revealed a richely vascularized lipomatous tissue traversed by nerve fibers. The surgical indication for such rare lesion is discussed.