Teratomas are tumors originated from mature or immature tissues. Teratomas are made up of one embryonic layer or all three embryonic layers (endoderm, mesoderm, or ectoderm). Herein we report a 41-year-old man who presented with vertigo, vomiting, and tinnitus. After physical examination, laboratory evaluation and performing computed tomography, cholesteatoma was diagnosed, however, during radical mastoidectomy a cystic tumor was found. The result of pathology proved middle ear and mastoid teratoma.