Objective To explore the etiology, clinical features, imaging characteristics and therapeutic regimen of cerebral venous sinus thrombosis (CVST). Methods One case of CVST mainly featured with isolated headache was retrospective analyzed, and related literature was reviewed. Results A 28-year-old male patient was admitted to the hospital with a 10-day headache and aggravated for 4 days. Magnetic resonance imaging (MRI) plain scan and enhancement of the head indicated filling defect of the left sigmoid and transverse sinuses, T1 and Flair showed high signal. The thrombosis of left transverse sinus and sigmoid sinus was confirmed by digital subtraction angiography (DSA). The patient recovered well after anti-infection and anticoagulant treatment. A total of 145 CVST patients with complete data in recent 5 years were collected, the most common features, pathogenic factor, disease location were headache accompanied by focal impairment symptom of neurologic system, hyperhomocysteinemia, superior sagittal sinus (SSS) and transverse sinus, respectively.Conclusion CVST patients normally exhibit the symptoms including the headache, hypopsia, limb weakness, epilepsy and disturbance of consciousness, only a few show simple headache. Anticoagulant therapy with vascular intervention is effectively capable of improving the prognosis of CVST patients. It is necessary to pay high attention to the patients with acute single recurrent progressive headache to avoid missed diagnosis and adverse consequences.