A review of patients with cholesteatoma at the Children's Hospital of Philadelphia from 1981 to 1986 yielded 161 children. Analysis of data from both outpatient and hospital charts revealed a higher incidence of males to females, and the peak incidence of cholesteatoma appeared to be in the 6-10 year age group. Otorrhea was the most common symptom; and on physical examination, the posterior-superior quadrant was most often affected. Both intact canal wall and open cavity procedures were employed depending upon the site and extent of disease. Seventy-six percent of patients underwent an intact canal wall or middle ear procedure initially, with the remainder requiring an open cavity procedure. Recurrent or residual cholesteatoma was found in 32% of 148 patients at the second procedure, and in 7% of 148 patients if a third procedure was necessary. Pre- and post-operative audiometric findings for 91 patients revealed 21% with decreased hearing post-surgically, 50% with no change in hearing and 29% with improved hearing. Post-operative complications were minimal.