Spontaneous cerebrospinal fluid rhinorrhea represents a distinct clinic entity that is likely a variant of idiopathic intracranial hypertension (IIH). Patients with spontaneous cerebrospinal fluid (CSF) leaks are generally middle-aged obese women with radiographic evidence of skull base defects, associated meningoencephaloceles, and empty sella syndrome, a common sign of increased intracranial pressure. Significant overlap exists in the characteristics of patients with spontaneous CSF leak and IIH. Endoscopic repair of the CSF fistula is the gold standard treatment for this condition, but emerging evidence supports the reduction of CSF pressure as an important adjuvant treatment in this patient population.