Conclusions. In congenital stenosis of the external auditory canal (CSEAC) with cholesteatoma, the bony wall of the external auditory canal (EAC) is most commonly involved. This involvement will lead to bone erosion of the EAC and may subsequently lead to the formation of postaural or cervical sinuses. High-resolution computed tomography (HRCT) of temporal bone can show characteristic signs of soft tissue mass in EAC, with adjacent bone erosion. Objective. To investigate the clinical features, differential diagnosis and management of CSEAC with cholesteatoma. Patients and methods. The clinical information for 10 cases of CSEAC with cholesteatoma was retrospectively reviewed. Results. The patients' ages ranged from 4.75 to 22 years (average 12 years). The diameter of EACs was 2 mm. All 10 ears had a history of postaural fistulae or sinuses. Bone erosion of EAC was distinctly shown in HRCT of all cases, as well as soft tissue masses, which led to enlargement of the bony canals. All patients underwent canaloplasty; eight ears received hearing reconstructions at the same time. Cholesteatoma in EACs was confirmed during the operations, accompanied by compression and destruction of the post-superior and/or inferior bony wall. Postoperative pathologic examinations proved the diagnosis of cholesteatoma, and excluded any tissue of bronchial cleft cyst or fistula. After a follow-up of 1-3 years, no recurrent cholesteatoma was found in any of the 10 cases. All reconstructed EACs were clean and smooth. The hearing levels in the eight ears that received hearing reconstructions increased 20-35 dBHL.