Spontaneous cerebrospinal fluid (CSF) rhinorrhea, occurring in the absence of recent trauma, is a distinct clinical entity that can be difficult to diagnose and treat. The case records of 28 patients with this entity were reviewed retrospectively. Pertinent historical data and the results of radiographic evaluation are discussed. The patients were classified into one of three major etiological categories: congenital anomaly, delayed posttraumatic, and tumor groups. The fistulas causing the rhinorrhea were approached extracranially or intracranially, depending on the suspected cause and location. A cure was obtained in 22 of the 27 patients surgically treated (81%). Seventeen patients were cured by a single operation, 5 were cured after multiple operations, and 5 continued to leak despite surgical treatment. Three of the 5 patients who were surgical failures had intracranial tumors, and 2 of them died from complications of persistent fistulas. With the use of a multidisciplinary team approach, an extracranial operation (transethmoidal or transsphenoidal) to repair a spontaneous CSF leak offers a high success rate and is associated with minimal morbidity in appropriately selected patients.