KEY POINTS 1. Congenital cholesteatoma (CC) is a relatively uncommon condition, accounting for 2-5% of all cholesteatomas. 2. Endoscopic ear surgery is commonly employed for limited cholesteatomas , usually Potsic stage 1 and 2. 3. This is the first reported case in the literature utilized a transcanal endoscopic ear surgery approach for an 11-year-old child with POTSIC 4 Congenital cholesteatoma, ensuring complete cholesteatoma removal and subsequent area reconstruction. 4. Endoscopic approaches offer advantages in terms of visualization and minimizing the need for external incisions and postoperative scarring, CC, improving the quality of life.