Bilateral primitive cholesteatoma of external auditory canal with congenital stenosis

•Primitive cholesteatoma of external auditory canal (EAC) is very rare and little known disease.•There are no specific clinical symptoms which can lead to confusion with other EAC pathologies.•Its diagnosis is clinical, but in case of stenosis, evaluation requires very close radiological analysis.•The treatment is surgical and depends on the extent of the lesions.