Cholesteatoma are found almost exclusively in the middle ear and mastoid. Occasionally this disease is seen in the external ear canal. Spontaneous ear canal cholesteatoma can be detected by careful review of clinical presentations of recurrent purulent ear discharge and chronic ear dull pain, and otoscopic findings of a focal area of osteitis and bone sequestration surrounded by hard wax and keratin debris. Ear canal cholesteatoma can be induced by congenital and acquired canal stenosis or atresia as a sequelae of ear trauma, post-ear surgery, recurrent inflammation, ear canal mass (osteoma) etc.. Irreversible damage by diffuse cholesteatoma may have occurred as a result of difficult observations of the stenosed ear canal conditions and the lack of early symptoms and signs of this disease. In this report, 11 cases with ear canal cholesteatoma were reviewed. All of them were successfully treated by the correction of the underlying diseases and removal of the cholesteatoma. With thess 11 cases’ experience, we especially stress the importance of continuous follow-up and early correction of congenital and acquired canal stenosis on the prevention and treatment of ear canal cholesteatoma.