Q fever is a zoonotic disease caused by Coxiella burnetii, with diverse and non-specific clinical manifestations. Intracranial infection caused by Coxiella burnetii is rare and is often misdiagnosed and underdiagnosed, leading to poor prognosis in some patients. This article reports a case of central nervous system intracranial infectious vasculitis caused by Coxiella burnetii through diagnosis assisted by metagenomic next-generation sequencing (mNGS) technique, indicating that mNGS technique plays an important role in rapid diagnosis of Q fever. After early diagnosis and precise treatment, the prognosis of patient was improved significantly. On this basis, relevant literatures at home and abroad are reviewed to summarize the clinical manifestations as well as diagnosis and treatment experience of intracranial infection caused by Coxiella burnetii, providing reference for domestic and foreign peers.