Keratosis obturans and external auditory canal cholesteatoma (EACC) have previously been considered to represent the same disease process. However, review of the literature and our cases reveal these to be two different clinical and pathological processes. Keratosis obturans presents as hearing loss and usually acute, severe pain secondary to the accumulation of large plugs of desquamated keratin in the ear canal. External auditory canal cholesteatoma presents as otorrhea with a chronic, dull pain secondary to an invasion of squamous tissue into a localized area of periosteitis in the canal wall. The treatment previously recommended for both of these conditions has been conservative debridement of the external canal and application of topical medication. While this remains the treatment of choice for keratosis obturans, surgery may be required to eradicate EACC.