Few evidence-based guidelines are available for the treatment of adult medulloblastoma, an extremely rare disease. Therapeutic regimens, typically modeled following pediatric protocols, consist of surgical resection followed by radiotherapy with or without adjuvant chemotherapy. Because of the rarity of this disease in adults, any treatment undertaken is based mainly on small and retrospective studies. Unlike pediatric patients, adults with medulloblastoma have been treated according to risk-adapted therapeutic strategies in only a few prospective studies. Overall, approximately 30% of patients experience recurrence and die of disease-related causes. Although the patients could respond to second-line treatments, the prognosis of patients with recurrence remains dismal. An important challenge for the future will be the biologic characterization of medulloblastoma in adults, with the identification of specific genetic patterns of patients with different prognosis and different response to targeted treatments.