Chondrosarcoma of the skull base is a rare tumor and must be considered potentially lethal. There are high demands on imaging techniques, neurosurgical intervention and radiation therapy. We describe a case of chondrosarcoma solitarily located in the area of the petrous apex in an adult female person initially suffering from diplopia. Data sets of computed tomography (CT) and magnetic resonance imaging (MRI) were used to perform CT–MR image data fusion to support preoperative planning and intraoperative image guidance for computer assisted skull base surgery with the help of a navigation system.