Cholesterol granuloma of petrous apex is very rare. Moreover it is difficult to differentiate from tumors of the cerebellopontine angle. The pathogenesis of cholesterol granuloma is delayed drainage, hemorrhage or inappropriate ventilation. Histopathologic findings are characterized by foreign body giant cells in fibrous connective tissue and cholesterol crystals. The symptoms in patient with cholesterol granuloma of the petrous apex are induced by obstruction of eustachian tube or compression of cranial nerves and carotid artery. Cholesterol granuloma usually produces symptoms of trigenimal, facial, and abducens nerve palsy and may not affect the auditory nerve. The characteristic findings of MRI scan demonstrate central regions of an increased signal intensity on both the T1- and T2-weighted images. The surgical goals in the treatment of cholesterol granuloma of the temporal bone are drainage and permanent aeration. Cholesterol granloma of the petrous apex must be differentiated from cyst, infectious diseases, vascular anomalies, and tumors of cerebellopontine angle. We report a case of cholesterol granuloma of petrous apex produces right facial weakness, which was treated with mastoidectomy and middle fossa approach.