Middle ear cholesteatoma is a common otologic disease in children.Compared with adult,pediatric middle ear cholesteatoma show some unique features in pathogenesis and clinical presentation.Usually insidious in its early stage,pediatric middle ear cholesteatoma is relatively difficult to diagnose.Moreover,it is characterized with ex-pansive lesions and severe hearing impairment related with its aggressive destructive nature.This review aims to im-prove understanding of pediatric middle ear cholesteatoma from the aspects of pathogenesis,clinical characteristics,treatment strategies and prognosis,in order to improve its diagnosis and treatment.