Conclusions: The diagnosis of occult otogenic cerebrospinal fluid (CSF) leakage is challenging and it can easily be misdiagnosed. Some characteristics of clinical presentation can supply important clues and confirmed diagnosis should be obtained according to these clues and suitable imaging studies before meningitis develops. Different surgical techniques should be adopted to treat the CSF leakage according to different leakage etiologies, and good results can be obtained. Objective: The aim of the study was to evaluate the diagnosis and surgical treatment of occult otogenic CSF leakage, including the characteristics of clinical presentation, imaging studies, and operation methods in order to decrease the rate of misdiagnosis and obtain a good curative effect. Methods: We performed a retrospective review of 11 cases of CSF leakage that were all misdiagnosed and accompanied by meningitis, operated in our department from 2007 to 2012 after a mean follow-up of 3 years. In this context, the characteristics of clinical presentation, imaging studies, and management of CSF leakage were studied. Results: The CSF leakage had arisen traumatically (n = 9) or congenitally (n = 2). The medical history and special clinical presentation such as repeated otorrhea or rhinorrhea, fever, headache, and unilateral deafness can supply important diagnostic clues. Imaging studies including high-resolution noncontrast CT (HRCT), CT cisternography, and magnetic resonance imaging (MRI) are very important diagnostic methods. The surgical repairs were performed via a transmastoid approach (n = 8), packing the vestibule (n = 1) or a translabyrithine approach (n = 2). Recurrent leakage did not occur.