Medulloblastoma is one of the most common malignant tumours of central nervous system in children. It accounts about two people per million per year worldwide and burgeoning. More than 70% patients diagnosed with medulloblastoma predominantly age group below 10. Histological variants of medulloblastoma are recognized as classic, nodular-desmoplastic, large cell-anaplastic and medulloblastoma with extensive nodularity. Symptoms include a headache, general malaise, failure to feed, vomiting, clumsiness, and other presentations that mimic common and benign childhood pathologies seen in primary care. Study data suggested that an inverse correlation between high-stage disease and duration of symptoms. Currently, medulloblastoma is classified clinically into high risk and standard (average) risk depending upon factors solely clinical – age, metastases and resection. The treatment strategies for medulloblastoma are maximal safe resection (plus/minus CSF diversion), neuraxis radiotherapy and chemotherapy. Medulloblastoma is the first brain tumour to show the efficacy of chemotherapy in large prospective trials. Effective chemotherapy regimens remain elusive for almost all patients with high-grade cortical or brainstem gliomas and for most young patients with residual or metastatic disease of any histology. Conventional chemotherapeutic agents continue to be developed to reduce toxicity and/or improve efficacy. Recent advances in tumour biology have changed the emphasis onto novel agents that target molecular changes crucial for tumour proliferation or survival. The toxicity and efficacy of several of these novel agents are currently being assessed in children with brain tumours.