Cholesteatoma refers to a buildup of squamous epithelium in the middle ear. Although it’s not a tumor, it can erode bones as it grows, making early treatment crucial. There are two primary types of cholesteatoma: congenital and acquired. Congenital cholesteatoma occurs due to epithelial growth in the middle ear during embryological development, while acquired cholesteatoma is further divided into primary and secondary types. The disease typically presents with symptoms such as otorrhea and hearing loss. If not treated promptly, cholesteatoma can lead to serious complications, categorized as either intracranial (like meningitis, abscesses, and sigmoid sinus thrombosis) or extracranial (like facial nerve palsy or labyrinthitis). These complications require immediate and aggressive treatment. High-resolution computed tomography (HRCT) of the temporal bone is the preferred imaging method for cholesteatoma, as it accurately depicts bone erosions. In cases where differential diagnosis is challenging or intracranial complications are present, an MRI can provide useful additional information. The standard treatment for cholesteatoma is a surgical procedure known as a mastoidectomy. Depending on the extent of the lesion, either an open or closed technique may be used. The primary objective is to fully eliminate the inflammation, achieve a dry ear, and, if feasible, restore the patient’s hearing.