Objective:To investigate the clinical features, diagnosis, and treatment experience of congenital stenosis of an external auditory canal with external auditory canal cholesteatoma. Methods:The clinical data of 152 patients(153 ears) with congenital external auditory canal stenosis complicated with external auditory canal cholesteatoma treated in the Department of Otorhinolaryngology Head and Neck Surgery of Beijing Tongren Hospital affiliated to Capital Medical University from January 2009 to December 2019 were analyzed retrospectively, including the age of onset, clinical manifestations, signs, audiology, high-resolution computed tomography(HRCT) of the temporal bone, mode of operation and so on. According to the preoperative imaging findings, intraoperative findings,and pathological results, 152 patients with congenital external auditory canal stenosis with external auditory canal cholesteatoma were treated with canaloplasty and tympanoplasty while clearing the cholesteatoma. Results:All patients were followed up for 2-2.5 years, there was no recurrence of cholesteatoma, and the reconstructed external auditory canal was spacious. The hearing levels of 108 ears who underwent hearing reconstruction were significantly improved, and the average hearing threshold was reduced by 20-35 dB. Conclusion:The stenosis of the external auditory meatus is easy to be complicated with cholesteatoma of the external auditory canal,and the occurrence of cholesteatoma of the external auditory canal is directly related to the diameter of the external auditory canal meatus.But the time of occurrence of the cholesteatoma is not directly related to the diameter of the external auditory canal. Severe congenital stenosis of the external auditory canal with auricle deformity is easy to be missed and misdiagnosed due to retroauricular redness, swelling, and ulceration. For this kind of patient, cholesteatoma should be treated first, and then plastic surgery such as auricle reconstruction should be performed. Retroauricular incisions should be avoided to create conditions for auricle reconstruction in the future.