Cholesteatoma is a destructive lesion of the temporal bone that gradually expands and causes complications by erosion of the adjacent bony structures. Bone resorption can result in destruction of the ossicular chain and otic capsule with consecutive hearing loss, vestibular dysfunction, facial paralysis and intracranial complications. Surgery is the only treatment of choice. The etiopathogenesis of cholesteatoma, however, is still controversial. This review was designed to understand the reasons for these disparities and to reduce or eliminate them. Future studies focused on developmental, epidemiological, hormonal and genetic factors as well as on treatment are likely to contribute to further understanding of cholesteatoma pathogenesis.