Despite its frequency (third most common brain tumor in children), publications about ependymomas per year are three times less frequent than for medulloblastomas (120 vs. 330). The knowledge and the sophistication of the protocols are lagging behind those of medulloblastomas. Accordingly, this has limited the progress made for these tumors. The improvement of neurosurgery is probably the driving force improving the outcome of children affected with this disease in the last 20 years. The three main questions that remain for ependymomas are the following: (i) Which biomarkers are useful to understand the oncogenesis and prognosis? (ii) Is there a role for chemotherapy? (iii) What is the appropriate strategy at the time of relapse?The management of ependymomas is currently mainly based on surgery and radiotherapy because of the relative ineffi cacy of chemotherapy. At each step of the management, these two treatments have to be considered for an optimal care. Presently oncological management for all intracranial ependymomas is homogenous despite the recent fi ndings that ependymoma biology may largely differ by location. First-Line Treatment: SurgeryThe initial surgical management is aiming at complete removal of the tumor, if necessary in more than one step because it remains the principal risk factor for survival. Indeed, in case of incomplete resection, one should not proceed to irradiation. Risk of relapse is fi ve times higher in case of incomplete resection even after fulldose radiation therapy [ 1 ]. In the Saint-Jude series, presently the best results published so far, 5-year event-free survival is only 41 % (95 % CI = 18-64 %) in case of near-total or subtotal resection versus 81.5 % (95 CI = 73-90 %) in case of gross-total resection, p < 0.0001. Any attempt should therefore be made to obtain a macroscopically complete resection. In between two surgeries, there is no defi nitive indication for chemotherapy since most of the regimens tested have not shown many responses [ 2 ]. Pre-irradiation chemotherapy may compensate for an incomplete resection as it has been shown with a recently published phase II using a standard chemotherapy regimen based on cisplatin in patients with incompletely resected; in this report, patients with incompletely resected ependymomas treated with pre-irradiation chemotherapy had similar although slightly lower EFS than those with completely resected ependymomas treated with irradiation only [ 3 ]. In addition, some anecdotal reports have shown that second surgery may be facilitated by a course or two of