Cholesteatoma of the external auditory canal can be classified as primary or secondary. The cause of primary external auditory canal cholesteatoma remains unclear and may be related to long-term chronic inflammation of the external auditory canal and its relative narrowness. The main symptoms include ear stuffiness, hearing loss, and ear drainage in case of infection. It is distinguished from cerumen embolism because of its destructive nature. Early external auditory canal cholesteatomas are small and can be cleaned regularly in an outpatient setting and treated locally. Larger external auditory canal cholesteatomas may be associated with destruction of the surrounding bone, invasion of the middle ear mastoid and even the facial nerve, and require hospitalization. Secondary cholesteatoma of the external auditory canal usually has a history of trauma to the ear, followed by narrowing or atresia of the external auditory canal and secondary cholesteatoma, which must be treated as soon as possible.