Posterior reversible encephalopathy syndrome (PRES) is a neurological disorder characterized by seizures, altered mental status and visual disorders, along with characteristic radiological findings. It is strongly related to hypertension induced by steroids and other immunosuppressive agents. There are an increasing number of reports regarding PRES arising during the course of chemotherapy for hematological malignancies. To clarify the risk factors for this phenomenon, we retrospectively analyzed pediatric patients undergoing treatment for hematological malignancies. Of 161 patients, six patients (3.7%) developed PRES with characteristic clinical and radiographic findings. Univariate analysis revealed that tumor lysis syndrome (TLS) was a significant risk factor for the onset of PRES. TLS is a significant risk factor for the development of PRES in pediatric patients receiving chemotherapy for hematological malignancies.