External ear canal cholesteatoma (EECC) is a rare otologic entity. Erosion of the inferior canal wall and accumulation of keratin debris are consistent findings. In the past there had been confusion between EECC and keratosis obturans, and they were thought to represent the same disease process. Currently, based on clinical and pathologic findings, it is believed that they are two different entities. In this article we present our experience in treating eight patients with EECC. For limited lesions, local debridement and curettage of necrotic bone is effective management. For more extensive lesions, canalplasty or tympanomastoidectomy is indicated.