OBJECTIVE: Intracranial and intraspinal clear cell meningiomas (CCMs) are rarely reported because of their extremely low incidence, and the current understanding of CCM is poor. The purpose of this study was to analyze the incidence and the clinical, radiologic, pathologic, and prognostic features of intracranial and intraspinal CCMs. METHODS: Among 14,310 cases of intracranial and intraspinal meningiomas that were surgically treated between 2006 and 2016 at Beijing Tian Tan Hospital, 56 were chosen for analysis and retrospectively reviewed. To determine which parameters were associated with longer progression-free survival (PFS) and overall survival (OS), statistical analysis was performed. RESULTS: CCMs accounted for approximately 0.39% of all intracranial and intraspinal meningiomas. Patients with CCM had a mean age of 32.3 years and there was a female predilection (20 males and 36 females). Gross total resection was achieved in 35 cases, and subtotal resection was achieved in 21 cases. All patients were followed up for 10-206 months after surgery. Twenty-six patients experienced tumor recurrence, and the median PFS was 48.0 months. The 1-year, 3-year, and 5-year PFS was 87.5%, 59.8%, and 41.8%, respectively. Twelve patients died of tumor recurrence, and the median OS was not available. The 1-year, 3-year, and 5-year OS was 98.2%, 91.3%, and 65.8%, respectively. Univariate analysis showed that total tumor removal was significantly associated with a better prognosis. Multivariate analysis confirmed only Simpson grade III and IV resection as an independent risk factor for shorter PFS. Radiotherapy mildly improved PFS after both gross total resection and subtotal resection, showing no significant difference because of the small sample size and short follow-up duration. CONCLUSIONS: CCM is a rare subtype of World Health Organization grade II meningioma. CCM typically involves young patients and shows a female predilection and high recurrence rate. When possible, total resection is the primary and most suitable treatment for CCM. For patients with primary tumors, radiotherapy is recommended after the initial operation regardless of the extent of resection. For patients with disease recurrence, secondary surgery combined with radiotherapy might serve as an effective treatment.