Posterior reversible encephalopathy syndrome (PRES) is a condition clinically characterized by headache, altered mental status, seizures, and visual loss and may be associated with systemic hypertension, preeclampsia/eclampsia, chemotherapy, immunosuppressive therapies in the setting of organ transplantation, and uremic encephalopathy. While brain imaging in patients with PRES typically reveals symmetric vasogenic edema within the parietal and occipital lobes, PRES may present with atypical imaging findings such as central brainstem and deep gray involvement without subcortical edema, and even spinal cord involvement. Additionally, PRES may be complicated in some cases by the presence of cytotoxic edema and hemorrhage. This review will serve to summarize the pathophysiologic theories and controversies underlying PRES, imaging features encountered in atypical and complicated PRES, and the implications these findings may have on patient prognosis.