OBJECTIVES: Cholesteatoma is the accumulation of exfoliated keratin within endocranium, mostly occurring in the middle ear or penumatization temporal bone area. It is classified as congenital and acquired. The annual incidence is around 3 per 100,000 children, mostly boys. It is aimed at reporting a case of acquired Cholesteatoma (assumed) in the left middle ear and mastoid in preschool attended at the Clinic of Otolaryngology and correlate with the scientific literature. CASE REPORT: A female patient, 4 years old, accompanied by his mother complaining to exacerbation of chronic othorrea bilaterally 2 weeks ago, otoscopy with bilateral purulent fetid, with central perforation in the left MT. After several attempts at treatment with oral and topical antibiotics as well as measures of hygiene and protection of the ears, there was persistence of fetid purulent othorrea in the left ear is necessary to accomplish mastoid computerized tomography showed that material with a soft tissue mass partially filling the most the left mastoid cells and skin thickening of the CAE. Were performed and treatment with intravenous antibiotics and left timpanomastoidectomy of closed cavity with ossicular chain reconstruction. Histopathology showed chronic inflammation consistent with Cholesteatoma. At 6 months postoperatively, MT has integrity, without othorrea and hearing recovery as operative audiometry. CONCLUSION post: We sought to highlight a rare presentation of otitis media Cholesteatoma in the left ear in preschool, which started early clinical presentation with symptoms suggestive suppurative otitis media of difficult clinical management and is delivered early and invasive, avoiding complications and therapeutic success.