Tuberculous otitis media, caused by Mycobacterium tuberculosis, is a chronic bacterial infection that affects the middle ear structures. Diseases with polymorphous and insidious clinical features can make diagnosis difficult and delay treatment. Herein we report a case of chronic tuberculous otitis media associated with cholesteatoma, highlighting the main factors that led to the diagnosis. Further, this report aimed to highlight the particularities in the clinical and surgical treatment of the case, given the association with chronic cholesteatomatous otitis media. This descriptive research, with qualitative analysis, was carried out at the Department of Otorhinolaryngology of the Hospital de Reabilitacao de Anomalias Craniofaciais in Sao Paulo, Brazil. This study highlights the importance of investigating tuberculosis as a differential diagnosis of granulomatous diseases of the middle ear and temporal bone, given the risk of serious and irreversible complications as the disease progresses.