Cholesterol Granulomas of the Petrous Apex (CGPA) are frequently misdiagnosed because of their variable and initially subtle clinical manifestations. Diagnosis of these lesions include computed tomography and magnetic resonance imaging. The authors report an interesting case of a giant cholesterol granuloma of the petrous apex in a 37 year-old male with tinnitus and moderate hearing loss. Clinical and histological features, diagnosis and surgical treatment will be discussed.