The study includes 54 cholesteatomatous ears in 50 children aged 16 years or less. The mean follow-up period after surgery was 7.1 years. In 26% of the ears the cholesteatoma was 'huge' involving the middle ear and the attic and filling the entire mastoid air cell system. A patient had lateral sinus thrombophlebitis. Patients with large cholesteatomas underwent canal wall down mastoidectomy with simultaneous tympanoplasty and, in most cases, cavity obliteration. Limited cholesteatomas were removed using either intact canal wall mastoidectomy or tympanotomy approach. Recurrence rate (including both residual and recurrent cholesteatomas) for the total series was 15% and 12% for the 50 cases undergoing one-stage surgery. Serviceable hearing (< or = 30 dB) was achieved in 57% of the ears. A reoperation was necessary in 26% and a third operation in 2%. At last follow-up examination, 94% of the ears had intact tympanic membranes but 4 patients (8%) suffered from cavity-related problems. Possible reasons for the disappointing results of surgical treatment for childhood cholesteatoma are discussed.