In order to investigate the early diagnosis and proper management of the cholesterol granulomatous tympanomastoiditis, 6 cases verified by pathology and surgery were reviewed. All of these cases had a longterm or a historical otitis media. Among them, different clinical features were presented, such as chronic serous otitis media, idiopathic blue eardrum, or as a primary occupying lesion of the middle ear. Depend on the extension of the disease, surgical procedure varied in the 6 cases, including exploratory tympanotomy, ventilation tube insertion, antroatticotomy, simple mastoidectomy, and radical mastoidectomy. Postoperative follow up for at least one--7 year showed no recurrence, and hearing improvement in 5 cases. The clinical basis of the diagnosis and management of this disease were also discussed.