In this review article the clinical manifestations, imaging findings, diagnostic and therapeutic approaches for intracranial hypotension are described. The typical manifestation, orthostatic headache, may sometimes be masked by atypical manifestations including coma, frontotemporal dementia and symptoms associated with leptomeningeal hemosiderosis. Spinal and cranial magnetic resonance imaging (MRI) findings are not always unequivocal and the diagnostic and therapeutic approaches are controversially discussed: Searching for the underlying spinal leak(s) of cerebral spinal fluid (CSF) is considered to be unnecessary or done with different modalities, such as computed tomography (CT) myelography, gadolinium-enhanced myelography and digital subtraction myelography. Various treatment approaches including conservative therapy, blind, fluoroscopy-guided and CT-guided epidural blood patches, CT-guided fibrin injection and surgery exist.