Cerebral venous sinus thrombosis (CVST) rarely occurs in children but has a mortality rate as high as 20%-78%. Because the clinical manifestation of this disease has no specificity it is easy to be misdiagnosed. This paper reported a case of CVST and reviewed the relevant literatures regarding to the pathogenesis, clinical and pathological features, diagnosis and treatment. The patient (male, 8 months old) was referred to the Beijing Children's Hospital with paroxysm headache and after vomiting for 50 days and having double visions for 2 days. He was definitely diagnosed with CVST by magnetic resonance imaging (MRI) and magnetic resonance venography (MRV). His neurological symptoms were improved after receiving intravenous urokinase and hormonal therapy for three weeks followed by oral anticoagulation. It is concluded that headache and papilledema are the most frequent symptoms in CVST and that MRI and MRV may be primal methods for the diagnosis of CVST.