Often described as a clinico-radiological entity, posterior reversible encephalopathy syndrome (PRES) is being increasingly diagnosed nowadays. However, mystery still surrounds its exact etiology. Though there are no standardized diagnostic criteria for this syndrome, there is a consistent feature associated with it: brain vasogenic edema in combination with neurotoxicity. The nonspecific nature of this condition leaves room for the diagnosis to be overlooked, leading to delays in providing appropriate treatment and unfavorable patient outcomes. PRES is associated with a variety of medical conditions including hypertension, eclampsia, autoimmune conditions, renal failure, sepsis, and an immunocompromised state, such as that secondary to the use of immunosuppressive therapy, human immunodeficiency virus (HIV), and organ transplants. Treatment by a multidisciplinary team and prompt identification and reversal of the underlying cause can lead to beneficial outcomes, as in the case we present in this report.