In an autopsy-based, case-control study of meningiomas over a 20-year period, there were 76 patients with meningiomas, and 20 of them also had an extraneural primary malignancy. In two control populations with autopsy-proved central nervous system (CNS) pathology (group I) and non-CNS pathology (group II), extraneural primary malignancies were seen in two patients in group I and in seven patients in group II. There was a high degree of statistical association between meningioma and extraneural primary malignancy (chi2 = 17.22, p less than 0.005 group I and chi2 = 7.61, p less than 0.01 group II). Screening of patients with meningioma for extraneural primary malignancy, and of patients with extraneural primary malignancy for meningioma are strongly indicated for the benefit of the patients as well as for further elucidation of the etiologic and temporal relationships between the two conditions.