PURPOSE OF REVIEW Spontaneous intracranial hypotension is a disorder caused by spinal CSF leakage. This article reviews the clinical presentation, diagnosis, and treatment of spontaneous intracranial hypotension. RECENT FINDINGS The hallmark symptom of spontaneous intracranial hypotension is acute orthostatic headache; however, clinical presentations can be heterogeneous. New evidence shows that lumbar puncture is not always necessary or sufficient to establish the diagnosis. Some patients may have normal opening pressure, which suggests that insufficiency of CSF volume (hypovolemia) rather than CSF pressure might be the underlying mechanism. Several neuroimaging modalities can aid in diagnosis and localization of the CSF leakage, including brain MRI, spinal MRI, CT myelography, digital subtraction myelography, and radionuclide cisternography. Complications, such as subdural hematoma, can lead to a change in the headache pattern and potentially life-threatening consequences. Conservative treatments, such as fluid supplementation, can provide temporary relief; however, epidural blood patches, especially targeted ones, are more effective and definitive. For patients with refractory spontaneous intracranial hypotension, surgical repair of spinal CSF leakages should be considered. SUMMARY Brain and spinal MRIs are important for the diagnosis and treatment of patients with spontaneous intracranial hypotension. Early treatment with epidural blood patches may be considered to shorten the disease duration and minimize the potential risk of complications.