<p>Congenital middle ear cholesteatoma (CC) is a condition of particular interest to otorhinolaryngologists due to its diagnostic and therapeutic challenges. Initially considered rare, its reported incidence has increased over the years, largely due to heightened awareness among practitioners and advances in diagnostic tools. The aim of this study was to describe the clinical characteristics, management strategies, and long-term outcomes of children treated for CC. This retrospective study included 12 children under the age of 16 who underwent surgery for CC in the ENT department of La Rabta Hospital over a 28-year period (1996–2024). The mean age of the patients was 11 years, with a sex ratio of 1. Hearing loss was the main complaint, reported in all cases. Diagnosis was established by identifying a whitish retrotympanic mass in 9 patients (75%). A preoperative diagnosis of CC was made in 9 patients—based on imaging in 7 cases and by paracentesis in 2 cases. All patients underwent surgical treatment. Ossicular erosion was found in 11 patients. According to Potsic’s classification, the disease was stage III in 50% of cases and stage IV in 50%. A closed type was observed in 7 cases, and an open type in 5 cases. Tympanoplasty with posterior cavity exploration was performed in 6 patients: a closed technique in 5 cases and an open technique in 1 case. Residual cholesteatoma was observed in 4 patients, identified via second-look surgery in 1 case and postoperative CT scan or MRI in 3 cases. Congenital middle ear cholesteatoma should be considered in patients with conductive hearing loss and an intact tympanic membrane. Early diagnosis and appropriate management are essential for a favorable prognosis. Imaging plays a pivotal role in diagnosis and long-term follow-up.</p>