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Posterior reversible encephalopathy syndrome (PRES) is a rare clinicoradiological entity that is an increasingly recognized complication of pediatric cancer treatment. However, there have been very few reports of PRES in association with non- Hodgkin′s lymphoma (NHL) and reported incidence is only 0.06%. The available literature and case reports on PRES were studied in depth, and the important conclusions were taken into account for writing athe review. We describe a case of PRES in a boy of 15 years with NHL on cyclophosphamide, doxorubicin, vincristine and prednisolone combination chemotherapy who presented with generalized seizures and reversible blindness. Magnetic resonance imaging of the brain revealed characteristic findings of the syndrome establishing the diagnosis. However, despite transient improvement of the symptoms, the patient succumbed to respiratory failure. The authors conclude that PRES is a rare entity especially in NHL patients that must be recognized early and should be managed aggressively as it may prove to be fatal also.