The terms posterior reversible leukoencephalopathy, reversible posterior cerebral edema syndrome, and posterior reversible encephalopathy syndrome (PRES) all refer to a clinicoradiologic entity characterized by headaches, confusion, visual disturbances, seizures, and posterior transient changes on neuroimaging. Clinical findings are not sufficiently specific to readily establish the diagnosis; in contrast, magnetic resonance imaging pattern is often characteristic and represents an essential component of the diagnosis of PRES. Typical lesions predominate in the posterior white matter, with some involvement of the overlying cortex; are hyperintense on T2-weighted images; and are usually hypointense or isointense on diffusion-weighted images, with an increase of the apparent diffusion coefficient, indicating vasogenic edema. The pathogenesis is incompletely understood, although it seems to be related to the breakthrough of autoregulation and endothelial dysfunction. Since its initial description, this syndrome has been subsequently described in an increasing number of medical conditions, including hypertensive encephalopathy, eclampsia, and the use of cytotoxic and immunosuppressive drugs. The diagnosis has important therapeutic and prognostic implications because the reversibility of the clinical and radiologic abnormalities is contingent on the prompt control of blood pressure and/or discontinuing the offending drug. On the contrary, when unrecognized, conversion to irreversible cytotoxic edema may occur.