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Malignant small round cell tumor is very rare, especially in the head and neck area. It is also difficult to make a differential diagnosis due to their undifferentiated or primitive character. Immunohistochemical staining and chromosomal study is useful to categorize these tumors. Since these are rare tumors, treatment protocols are is not well established. While combined treatments (surgery, chemotherapy, radiotherapy) is currently being applied, the tumor still has a poor prognosis. We present a rare case of a rapidly growing temporal bone malignant small round cell tumor which initially showed facial paralysis.