Learning Objectives: Congenital cholesteatoma surgical technique preserving intact tympanic structures.

Introduction: Congenital cholesteatoma is often presented as an asymptomatic disease. It is usually discovered during the otoscope examination, seen as a white mass behind a normal intact tympanic membrane. The mean age of presentation is in children between 5 and 10 years old. The early diagnosis and treatment is essential in order to avoid future complications. In children the minimally invasive approach is essential preserving the anatomic ear structures.

Clinical case: We present a minimally invasive approach for congenital mesotimpanic cholesteatoma. We perform an endoaural approach, with two incisions, upper and lower one, and a conchomeatal flap is made. This allows a direct approach to the middle ear. The posterior and anterior annulus are detached extending the anterior annulus 90 degrees anterior to the short process of the malleus, maintaining the stability of the tympanic membrane in the umbus. The ossicular chain remains intact. The cholesteatoma is removed and it is checked tby endocopic vision the full excision of the matrix.

Conclusions: We present a minimally invasive endoaural approach to reach full control and elimination of a desease that left to its natural evolution can develop intracraneal and extracraneal complications.