Intracranial hypotension syndrome is an essential or, more frequently, secondary entity. This is characterized by orthostatic headaches and diffuse dural gadolinium enhancement on magnetic resonance imaging with or without subdural fluid collections or evidence of descent of the brain. What determines the various clinical and imaging features of intracranial hypotension syndrome is the cerebrospinal fluid volume depletion. In this article, we review the clinical and neuroimaging findings, pathophysiology, and treatment of this syndrome.