PURPOSE: To describe a patient with classic presentation of spontaneous intracranial hypotension and subsequent improvement with targeted epidural blood patch. METHODS: Report of one case and review of the literature. RESULTS: Examination of cerebrospinal fluid after lumbar puncture disclosed a reduced opening pressure, an increased level of protein, and lymphocytic pleocytosis. Magnetic resonance imaging of the brain with gadolinium showed diffuse enhancement of the pachymeninges, no evidence of leptomeningeal enhancement, and chronic subdural fluid collection. Radionuclide cisternography demonstrated reduced activity over the cerebral convexities, early accumulation of radiotracer in the urinary bladder, and direct evidence of leakage at the cervicothoracic junction (C7-T1). Clinical, laboratory, and radiologic features were consistent with the diagnosis of spontaneous intracranial hypotension. Therapy with a targeted epidural blood patch resulted in the rapid resolution of symptoms. CONCLUSIONS: In this report, we describe a classic case of spontaneous intracranial hypotension in a 63- year-old man with an initial presentation of postural headaches, blurred vision, pain in the left eye, diplopia on left gaze, and neck soreness.