Spontaneous cerebrospinal otorrhea (SCSFO) from tegmen or posterior fossa defects is uncommon. Twenty-nine cases have been detailed in the literature to date. We report an additional four cases from three patients. This group of 33 cases of SCSFO from tegmen or posterior fossa defects is characterized by certain clinical features. These patients are usually older adults (mean age 48 years, range 8 months to 80 years). Aural fullness with a serous appearing middle ear effusion, or clear otorrhea, often subsequent to tube myringotomy, are the usual initial symptoms. Meningitis was the initial manifestation in eight patients (24%), and ultimately occurred in an additional four patients (12%). The pathophysiologic basis for SCSFO from tegmen defects is discussed. Methods for diagnosing and treating CSF otorrhea from tegmen defects are reviewed.