The incidence of cholesteatoma in children living in an urban area of western Sweden has been studied. The total population of this area is 470,000 out of which 100,000 are children, 16 years or younger. The charts of all the children undergoing cholesteatoma surgery during the time period from 1977 to 1986 were collected and analyzed. Only those patients who had not been exposed to cholesteatoma surgery before were included. In a follow-up study the clinical situation and hearing level have been evaluated 4 years or more after surgery. Nineteen patients, that is an incidence of 0.4 children out of 100,000 inhabitants each year, were found meeting these criteria with an irregular distribution over the 10 year period. During the first two years 11 cases were found, but in the following 8 years only 8 new cases were identified. Intact wall tympanoplasty was used in 10 cases and canal wall down operation in 9 cases. Residual cholesteatoma was detected in 6 cases and recurrent cholesteatoma in one case. Six of these had an intact wall tympanoplasty and all but one were later reoperated with a canal wall down approach. The study reveals a decrease in the incidence of cholesteatoma in children over a 10-year period. The more frequent use of tympanometry and otomicroscopy is a possible explanation.