Cholesteatoma occurs in 10% of cases of chronic otitis in children. In most children, the clinical form is very similar to acquired cholesteatoma in the adult. However, we observed certain clinical variations in our retrospective series of 80 cases seen in our unit over the past 8 years. In most all cases, otorrhoea and hypoacousia were the presenting signs. The tympanic membrane was fully intact in 10% of the cases raising the possibility of congenital pathogenesis. An analysis of the correlations between per-operative observations and tomodensitometric results was conducted. Closed tympanoplasty, with a second operation 11 months later, was performed in 84% of the cases. Residual cholesteatoma was observed in 41% of the second operations and relapse occurred in 16%. These anatomic and functional findings, compared with those in the literature, demonstrate that no major difference in the published series. Presently the most important point is to separate acquired and congenital forms, which differ in terms of pathogenesis and clinical presentation, but are treated in the same manner. Progress in the treatment of cholesteatoma of the middle ear in children will come from prevention through earlier diagnosis of pathological manifestations in the ear nose and throat, in particular blocked Eustachian tubes, and through follow-up and treatment of pre-cholesteatomous states, as well as further advances in fundamental research.