A case of anaplastic oligodendroglioma with femoral metastasis is presented in a 37-year old male with a 2-year history of progressive headaches and dizziness associated with a 2-month history of epilepsy and right hemiparesis. Magnetic resonance imaging (MRI) demonstrated a solid temporoparietal tumour and the patient underwent a left temporal craniotomy and subtotal resection followed by limited-field radiation therapy. The pathological diagnosis was anaplastic oligodendroglioma. The patient presented with left hip pain 3 years later. Radiography and computed tomography demonstrated osteosclerosis of the left proximal femur, and MRI revealed an intramedullary metastatic lesion. Total body (99m)Tc-methylene diphosphonate bone scan showed hyperactivity in the lesion and open biopsy confirmed it was a metastasis from the cerebral oligodendroglioma. The patient was treated with temozolomide and, to date, there is no sign of recurrence or progression in either the brain or the femur. Seven previously reported cases of extracranial skeletal metastasis from anaplastic oligodendroglioma are reviewed. Co-deletion of chromosome arms 1p and 19q and O(6)-methylguanine DNA methyltransferase status remain the most important prognostic and predictive markers.