Valiakhmetova A, Gorelyshev S, Konovalov A, et al. - Researchers describe a case of glioblastoma in a 3-year-old girl who continued to exhibit an exceptional and durable response (> 2 years) to olaparib, the poly (ADP-ribose) polymerase inhibitor. Presentation with persistent and progressive seizure activity was attributable to a large heterogeneously enhancing, mixed cystic and solid mass in the left frontal-parietal-temporal region. Glioblastoma was confirmed on the histopathologic study of resected tumor tissue. No BRAF or H3F3A mutations were present, as revealed by comprehensive genomic profiling. Standard-of-care treatment with radiation and temozolomide was given following debulking surgery. After 9 months, the patient was treated with olaparib in combination with temozolomide for 16 cycles. The patient could well-tolerate this regimen and had a reduction in tumor size on serial imaging. Since completion of the regimen, the patient continued to be neurologically intact with no proof of tumor recurrence. This is the first case report wherein a therapeutic strategy involving olaparib elicited a maintained durable response in pediatric glioblastoma. The potential clinical usefulness of including these agents into the treatment of pediatric high-grade gliomas was more generally emphasized by the findings.