患儿，男，14岁，因无热抽搐后意识不清4 h入院。病前3个月曾患双眼葡萄膜炎。脑脊液白细胞轻度升高。头颅磁共振成像平扫及增强示双侧大脑半球多发异常信号，伴点状及结节样强化；磁敏感加权成像示双侧额叶、左侧顶叶病灶内多发点状出血，提示血管炎。脑活检结果示炎性肉芽肿性病变。患儿无继发性因素，最终诊断为肉芽肿性原发性中枢神经系统血管炎。患儿接受甲泼尼龙琥珀酸钠及霉酚酸酯治疗后病情好转。该文报道1例罕见的儿童肉芽肿性原发性中枢神经系统血管炎，为该病的诊断及治疗提供经验。

A 14-year-old boy was admitted to the hospital due to a single episode of afebrile seizure and four hours of impaired consciousness. Three months prior to admission, he had a history of bilateral uveitis. Cerebrospinal fluid analysis revealed a mild elevation in white blood cell count. Cranial magnetic resonance imaging and contrast-enhanced scans showed multiple abnormal signals in both cerebral hemispheres, with punctate and nodular enhancement. Susceptibility-weighted imaging revealed multiple punctate hemorrhages within lesions in the bilateral frontal and left parietal lobes, suggestive of vasculitis. Brain biopsy demonstrated inflammatory granulomatous lesions. No secondary causes were identified, and the final diagnosis was granulomatous primary central nervous system vasculitis. The patient's condition improved after treatment with methylprednisolone sodium succinate and mycophenolate mofetil. This report describes a rare case of granulomatous central nervous system vasculitis in a child and provides valuable insights for the diagnosis and treatment of this disease.