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Chronic inflammatory tumor-like lesions of the temporal bone represent a difficult clinical task for the skull base surgeon. Their osteolytic aggressiveness endangers vital structures and may not be controlled by surgery alone. We present the course of four cases of fibroinflammatory pseudotumor of the temporal bone which were treated by a combined approach of skull base surgery and chemotherapy. Three patients were deafened by the disease and underwent several operafive measures. One patient was lost, most likely due to an arrosive bleeding of the internal carotid artery. The chronic and recurrent process could only be stopped by petrosectomy, followed by antiproliferative chemotherapy. Two patients were subsequently provided with a cochlear implant. The differential diagnosis, diagnostic, and operative options of this rare but severe disease are discussed.