Ependymomas are usually benign tumors of the central nervous system that derive from the ependymal cells lining liquor-filled spaces. Myxopapillary ependymomas are a variant occurring almost exclusively in the region of the cauda equina. Histologically, these tumors are benign (WHO grade I). The following case describes the first reported spread of a spinal myxopapillary ependymoma into the internal auditory canal. The symptoms and diagnostic results are similar to other tumors of this location. There are no typical findings in the diagnostic imaging (CT, MRI). The extended middle cranial fossa approach was chosen for surgical treatment. Adjuvant radiotherapy is indicated in cases of incomplete resection or recurrence.