Objective: In this study, congenital cholesteatoma cases that were diagnosed and surgically treated in our clinic are presented and discussed in the light of the current literature. Material and Methods: Clinical records of seven patients (four males and three females, aged between 6 and 49 years), operated for congenital cholesteatoma between 2003 and 2008 were reviewed. None of the cases had a history of previous surgical intervention or chronic otitis media. All patients were evaluated with audiogram, computerized tomography, and magnetic resonance imaging. Results: Five cases presented with a progressive conductive hearing loss, whereas one patient with tinnitus and another with ear pain. Otoscopic examination showed that tympanic membranes were intact in all cases. In two cases, a white mass causing bulging of the tympanic membrane was seen behind the eardrum. There was a hearing loss in all, but three cases. A canal wall up mastoidectomy was done on two patients, three patients had a modified radical mastoidectomy one patient had a transcanal atticotomy with ossiculoplasty, and one patient had a modified transotic approach. Four patients had stage three, two patients had stage four, and one patient had stage two congenital cholesteatomas. Postoperative early complications were not seen in any of the patient. Conclusion: Congenital cholesteatoma can be diagnosed after observation of a white colored mass behind the tympanic membrane either in patients presenting with hearing loss or during routine examination. However, in most patients, this finding cannot be observed. For this reason, especially in young patients with a normal looking tympanic membrane and a conductive hearing loss, the possibility of congenital cholesteatoma should be kept in mind.