Microtia with aural atresia accompanied with cholesteatoma is extremely rare condition. A 20-year-old female student with microtia in the right ear was treated surgically in total four times between 1981 and 1987. She was complaining of right otorrhea on September 21st 1993. The fruncle was found in the central portion of the reconstracted auricle in the right ear and did not respond to systemic antibiotics therapy. Then, CT and biopsy detected the existence of external autiory canal cholesteatoma in her right ear. The lesion was removed and no recurrence has been found to date.