A 53-year-old man presented with a rare meningioma associated with Werner syndrome. Screening brain magnetic resonance (MR) imaging with gadolinium had detected multiple homogeneously enhanced tumors in the right convexity and in the anterior and posterior thirds of the falx cerebri after surgery for osteosarcoma in his right leg at age 52 years. Ten months later, the right convexity tumor was removed because follow-up MR imaging detected tumor growth. The histological diagnosis was transitional meningioma. The postoperative clinical course was good and the patient remains healthy. Review of the literature found meningiomas associated with Werner syndrome occur about two times more frequently in men than in women, and typically in the fourth decade. Most meningiomas associated with Werner syndrome are benign, but are sometimes complicated with extracranial tumors such as sarcoma, thyroid carcinoma, and others. Patients with meningioma associated with Werner syndrome should be carefully followed up to detect the occurrence of other extracranial tumors such as sarcoma by brain MR imaging, echography, or body computed tomography.