Abstract: Objective To discuss the diagnosis, surgical indication, endoscopic fenestration procedures, and prognosis of symptomatic septum pellucidum cyst. Method Ten patients with symptomatic septum pellucidum cyst showing clinical symptoms of elevated intracranial pressure and epilepsy with the maximal cyst width ranging from 12 to 35 mm underwent endoscopic cystoventriculostomy. Results During the follow-up period, 8 patients were symptom-free, one had significant improvement of the symptoms, but one failed to respond to the treatment. Conclusion Endoscopic cystoventriculostomy is effective in the treatment of symptomatic septum pellucidum cyst, but care must be taken to avoid collateral injury in the operation.