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Stem cell transplantation has been performed for various diseases, contributing to a markedly improved prognosis in some cases. However, several complications, including posterior reversible encephalopathy syndrome, have been evident. Although posterior reversible encephalopathy syndrome was originally defined as a reversible disease, it has become clear that it is not reversible in all patients. Epstein-Barr virus causes a wide spectrum of neurologic disorders, including epilepsy. To our knowledge, Epstein-Barr virus was not previously reported to cause epileptic spasms. We describe a girl with epileptic spasms after posterior reversible encephalopathy syndrome associated with stem cell transplantation for chronic Epstein-Barr virus infection. Although direct correlation was not clarified, this is a rare case that may contribute to our understanding of the neurologic complications of stem cell transplantation for chronic Epstein-Barr virus infection.