Intracranial hypotension syndrome and cerebral venous thrombosis are both low incidence neurological diseases. The incidence rate of isolated cortical vein thrombosis is only about 5% of all cerebral venous system thrombosis. Intracranial hypotension syndrome complicated with isolated cortical vein thrombosis is very rare in clinic. This paper reports a case of intracranial hypotension syndrome complicated with isolated cortical vein thrombosis and seizures. The symptoms improved after rehydration， anticoagulation and antiepileptic treatment. In order to provide reference for clinical diagnosis and treatment of these diseases， relevant literatures were reviewed and summarized.