Posterior reversible encephalopathy syndrome (PRES) is one of the most common neurologic complications following hematopoietic stem cell transplantation (HSCT). We aimed to evaluate incidence, clinical, and imaging features of PRES in pediatric patients with FA following HSCT. This prospective study was carried out on all post-HSCT patients with underlying FA disease between 2014 and 2017. Brain CT scan and MRI were performed in all individuals who developed neurologic symptoms. The diagnosis of PRES had done based on clinic-radiological evidence. Follow-up MRI was carried out in all patients with PRES within two months. Forty-one patients with FA (28 males, mean age: 8.19±3.25 years) were enrolled. Out of 15 patients with acute neurologic symptoms, PRES was diagnosed in 9 individuals (21.95%). The occurrence of PRES was significantly higher among patients who had a donor with a one-locus mismatch (p-value: 0.02). Donor relation, stem cell source, and GvHD grading did not have any significant association with the development of PRES. MRI showed asymmetric vasogenic edema in five patients, overt infarct in one, and foci of micro-hemorrhage in three individuals that one of them developing hemorrhagic infarct. The patient expired shortly, while persistent micro-hemorrhage was noted on the other two patients. The result of our study demonstrated the risk of developing PRES after HSCT is higher in FA compared to other diseases reported in articles (21.95% vs. 1-10%), and in contrast to its term, it might be irreversible and has adverse effects on the results of HSCT. Increased vascular and endothelial fragility in FA may impact on the higher frequency of PRES in these individuals.Copyright © 2020. Published by Elsevier Inc.