Teratoma is a germ cell tumor, which is rare behind the ear. We described a rare case of retroauricular teratoma accompanied with congenital malformation of external and middle ear and cholesteatoma of middle ear in a 13-years-old girl. Congenital microtia and ankylotia of right ear was found since childhood, suppuration occurred repeatedly behind the right ear 1 year ago. Temporal bone CT and MRI scan revealed congenital malformation of middle ear and cholesteatoma of middle ear. Cystic mass containing a tooth was found intraoperatively. The pathological results showed that it was benign cystic teratoma. It showed no evidence of recurrence on the patient during 3 months follow-up.
