Oligodendroglioma (ODG) constitute a specific type of gliomas, with a better prognosis than astrocytic tumours of similar grade. No clear criteria exist to differentiate astrocytoma from ODG, leading to a significant inter-observer variation in the diagnosis of ODG. ODG are genetically characterised by chromosomal lesions in the 1p36 and the 19q13.3 regions. ODG tumours without these lesions but with TP53 lesions or glioblastoma multiforme-like genetic lesions (loss of chromosome 10 and amplification of 7p) probably constitute a different entity with an oligodendroglial phenotype. Recent clinical trials have shown that ODG are sensitive to chemotherapy with 60-65% of patients responding, with a median response duration of 1-1.5 years. This holds for both low and high-grade ODG; the response rate in mixed oligoastrocytomas may be slightly less but is still better than that of pure astrocytic tumours. There is a strong correlation between a favourable response to chemotherapy and the presence of 1p36 and 19q13.3 lesions, suggesting this may be used to select patients for treatment. The presence of 1p lesions is also related to a longer progression free survival after radiation therapy, implicating that the assessment of the presence of 1p lesions is an important prognostic tool for ODG. The chemosensitivity of ODG is not limited to the PCV schedule. Ongoing trials are investigating whether adjuvant chemotherapy after surgery and radiation therapy provides better survival and quality of life in newly-diagnosed anaplastic ODG. However, even patients with a good response to chemotherapy will ultimately relapse. This calls for improvement of the chemotherapy schedules and for improvement of second line chemotherapy. In the near future molecular analysis may become an important tool to identify tumours that are likely to respond to chemotherapy.