Congenital cholesteatomas arise from embryonic epithelial cell rests in the middle ear cleft. We report our series of 19 cases observed in 18 children between 1985 and 1990. Clinical data and surgical observations are analyzed. In regard to the location of the lesions, our findings are consistent with most of what has been reported in the literature. Sixty-eight percent of the lesions originated from the anterosuperior part of the mesotympanum. Only 10% were posterosuperiorly located. Pathogenic mechanisms are discussed with respect to the lesion's primary site.