The congenital cholesteatoma is a rare benign tumor whose diagnosis is mainly made during childhood. Otoscopic examination shows a white retrotympanic collection with or without hearing loss. In case of a late diagnosis, the disease will spread to the surrounding structures, leading to ossicules destruction, facial palsy, sensorineural hearing loss and vestibular dysfunction. Surgery is the therapeutic gold-standard, with different techniques chosen based on the cholesteatoma's extension. Patient should benefit from a long term medical and radiological (MRI) follow-up.