Cholesteatomas of mastoid origin often grow silently, remaining unidentified and untreated for years. Cholesteatomas may not even be detected until they are quite massive, and diagnosis may only occur as a result of incidental imaging. Here, we report a case involving a 59-year-old man with delayed presentation of a huge mastoid cholesteatoma complicated by skull base erosion and cerebrospinal fluid leakage. Regular otologic, audiologic, and imaging examinations are the best strategies to avoid undesirable consequences related to chronic otitis media with cholesteatoma. Early recognition of this rare comorbid condition is essential because appropriate and timely treatment can prevent it from becoming fatal.