Since the late 1970s, the author has had the opportunity to prospectively study, document, and surgically manage 40 cases of congenital cholesteatoma. All cases met strict criteria for inclusion in the study, all were surgically and pathologically confirmed, and were definitively followed. During that same period, 38 cases were managed by other members of the otolaryngology department. It is the purpose of this thesis to critically study this personal and institutional experience, and to validate the rationale for early diagnosis and prompt and effective surgical intervention.