BACKGROUND: Systemic treatment options are limited for meningioma that progress following surgery and radiotherapy. VEGF is associated with neovascularization, tumor growth, and edema in meningioma. This prospective phase II study was undertaken to determine the efficacy of bevacizumab (BEV), a VEGF ligand binding monoclonal antibody in patients with progressive benign (BM), atypical (AM), and malignant meningioma (MM) as measured by 6-month progression free survival (PFS-6). Secondary endpoints included median progression free survival (mPFS), median overall survival (mOS), and radiographic response rate (RR). METHODS: Patients with histologically confirmed BM, AM, or MM with radiographic progression were eligible. There was no limit on prior therapies. BEV was administered 10 mg/kg intravenously every two weeks. After 6 months of therapy, stable patients were eligible to switch to an every 3-week schedule of BEV (15 mg/kg). Clinical examination and imaging were performed every 4 and 8 weeks respectively for the first 6 months then every 6-12 weeks. Responses were graded using the Macdonald criteria. RESULTS: 40 patients were treated: 15 BM, 22 AM, and 13 MM. Median KPS was 80. Median age was 54 (23-81) years. Median number of BEV infusions was 16 (2-62). Best responses were stable disease (BM: 100%; AM: 86%; MM: 82%); partial response (BM: 0%; AM: 5%; MM: 0%), progressive disease (BM: 0%; AM: 5%; MM:18%). PFS-6, mPFS, and mOS were 87%, 22.5 months, 35.6 months for BM; 77%, 15.3 months, not reached for AM, and 46%, 3.7 months, 12.4 months for MM. CONCLUSIONS: Treatment of progressive meningioma with BEV commonly leads to disease stabilization. The results are promising compared to a contemporary meta-analysis, in which PFS6 was 29% and 27% for BM and AM/MM respectively, but require confirmation. Toxicity was minimal and expected for BEV notwithstanding prolonged drug exposure.