Medulloblastoma is the most common malignant brain tumors in children. Current management combines surgery, radiotherapy, and chemotherapy. Current treatment of medulloblastoma is based on a clinical risk-stratification system that takes into account age, extent of resection and metastatic status. High-risk medulloblastoma patients are defined by the presence of metastatic disease and/or an incomplete resection with a residual amount of tumour>1.5 cm2. This review describes the evolution in the management of high-risk medulloblastoma patients during recent 4 decades and recent changes in the definition of high-risk patients as a result of major advances in the understanding of the molecular heterogeneity of medulloblastomas.