Cholesteatoma of the external auditory canal (CAE) is a rare disease with an incidence of 1:1000 among patients who visit an otologic practice. This disease is characterized by an invasion of the squamous epithelium with erosion of a localized area within the bone canal. CAE is more commonly observed in the elderly, is usually unilateral, and causes insidious pain and mild hearing loss. It is difficult to clinically differentiate between CAE and its differential diagnoses, such granuloma of the external auditory conduct. The aim of this case report is to describe the clinical presentation and the conduction of this rare case and to compare it with the available literature. CASE REPORT: A 31-year-old while woman born in Rio de Janeiro presented at the Hospital Universitario Antonio Pedro with a slow-growing mass in her left external auditory canal along with ear fullness, otalgia, and otorrhea. Otoscopy revealed a mass of fibroelastic consistency occupying the entire external auditory canal. Computed tomography (CT) showed that the lesion was restricted to the auditory canal. Excisional biopsy was performed, and the histopathological analysis revealed a cholesteatoma of the external auditory canal, which was successfully removed. The audiometry test conducted after the procedure was normal. In conclusion, presentation of this case is important due to the rarity of this disease and because it occurred in a young woman. The patient was surgically treated and is currently under clinical observation due to the possibility of recurrence.