A very rare case of 46-yaer-old woman with chondromyxoid fibroma (CMF) of infralabyrinthine area of temporal bone was described in this article. The only manifestation of this disease was a severe temporary pain in the postauricular area with irradiation in the occipital bone and headache during the last 3 months. A detail description of the CT scan and MRI data was presented. Tumor removing was performed through the retrofacial approach with combination of the microscopic and endoscopic assistance technique, which allows to had a good visualization and controlling of tumor separation from the vital structures with hearing and facial nerve function preserve. Also, we presented a brief review of literature with differential diagnosis of the CMF of the temporal bone, which conducted to minimize the diagnosis mismatches in the otologic and head and neck practice and to optimize the treatment of patients with such tumor.