A case of spontaneous intracranial hypotension (SIH) caused by a cerebrospinal fluid (CSF) leak at C1-2 is described. The patient, a 46-year-old gentleman, presented to the emergency department with a severe, orthostatic neck pain and occipital headache of sudden onset. He was diagnosed with SIH and admitted, but failed to respond to conservative management. Imaging studies suggested that C1-2 was the spinal level responsible for the CSF leak, and he underwent a blood patch therapy delivered via an epidural catheter inserted from C6-7. His neck pain disappeared a day after the procedure, and he remains free of symptom for more than a year. SIH with a CSF leak at the upper cervical spine may be least amenable to conventional epidural blood patch delivered from the lumbar spine. Delivery of autologous blood patch via an epidural catheter inserted from the lower cervical spine can be a safe and effective method for such patients.