AbstractOf 325 previously untreated cholesteatomas, 109 were found to be in children 13 years old and younger—however, these 109 ears were not found to consist of one clinical entity. Sixty-three of the 109 ears presented a marginal perforation or a retraction pocket, at the level of Shrapnell's membrane or beyond the posterosuperior quadrant. In this group the cholesteatoma was distributed mainly in the attic and mastoid and was associated with a non-cellular mastoid. A second group comprising 31 ears presented with cholesteatoma behind an intact drum and were considered to be primary cholesteatomas. These were distributed mostly in the tympanic cavity as cystic epidermoid formations—their mastoid was usually pneumatized. Eight cholesteatomas were related to a central perforation. These ears presented features very similar to the primary cholesteatomas i.e., a pneumatized mastoid and tympanic cavity distribution. The similar features of this group make us think that they may have also originated as primary cholesteatomas which eventually perforated and bring the percentage of primary cholesteatomas in children to 38.3%. Seven of the 109 ears with cholesteatoma were of an indeterminate character.