As endoscopic instrumentation, techniques and knowledges have significantly improved recently, endoscopic ear surgery has become increasingly popular. Transcanal endoscopic ear surgery (TEES) can provide better visualization of hidden areas in the middle ear cavity during congenital cholesteatoma removal. We aimed to describe outcomes for TEES for congenital cholesteatoma in a pediatric population.Twenty-five children (age, 17 months to 9 years) with congenital cholesteatoma confined to the middle ear underwent TEES by an experienced surgeon; 13 children had been classified as Potsic stage I, seven as stage II, and five as stage III. The mean follow-up period was 24 months. Recurrence of congenital cholesteatoma and surgical complication was observed.Congenital cholesteatoma can be removed successfully via transcanal endoscopic approach in all patients, and no surgical complications occurred; only one patient with a stage II cholesteatoma showed recurrence during the follow-up visit, and the patient underwent revision surgery. The other patients underwent one-stage operations and showed no cholesteatoma recurrence at their last visits. Two patients underwent second-stage ossicular reconstruction.Although the follow-up period and number of patients were limited, pediatric congenital cholesteatoma limited to the middle ear cavity could be safely and effectively removed using TEES.