We report 7 patients, ages 21 to 49 years, with systemic metastasis from anaplastic oligodendroglioma. Metastases developed in the scalp, cervical lymph nodes, bone, and other organs 1 to 76 months after the most recent surgery and 18 to 86 months after diagnosis. Systemic metastases responded to focal radiotherapy or nitrosourea-based chemotherapy for 6 to 18 months. Five patients have died, 4 to 24 months after the appearance of systemic metastases, all with progressive cerebral and systemic tumor. We observed 2 distinct patterns of spread of oligodendroglioma. Pattern 1, initial scalp or regional lymph node involvement followed by distant metastasis, was associated with multiple craniotomies. Pattern 2, distant metastasis without scalp or regional lymph node spread, was associated with early radiotherapy and chemotherapy. Longer-than-expected survival was not essential to metastasis. We speculate that anaplastic oligodendrogliomas possess special characteristics favoring metastasis and that early aggressive treatment alters the biology of this disease.