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Ten rare cases of symptomatic septum pellucidum cysts in patients who underwent endoscopic fenestration are described. The approaches and techniques used in the management of these cysts and the endoscopic surgical indications are discussed.In the past 5 years, 10 patients (age range, 3–60 yr) with symptomatic septum pellucidum cysts underwent neuroendoscopic fenestration. The most common symptom was intermittent headache (seven patients) accompanied by dizziness, vomiting, and epileptic seizures. Two patients presented with epileptic seizures. One patient presented with abnormally increased head circumference. Magnetic resonance imaging scans of 10 patients showed septum pellucidum cysts, two with hydrocephalus, and two with pituitary microadenoma.All 10 patients underwent endoscopic fenestration with a rigid endoscope via a frontal approach. Eight cases were performed freehand. Two cases were assisted by a frameless neuronavigation system. Postoperatively, the mass effect of the cysts and the symptoms resolved immediately, and computed tomographic or magnetic resonance imaging scans showed significant decrease in the cyst size and no recurrence during follow-up. Ventricular sizes in the two patients with hydrocephalus were normal.Neuroendoscopic pellucidotomy could be an effective, safe, and convenient therapeutic method for symptomatic septum pellucidum cysts. This approach might provide communication between the cyst and the ventricular system, thus avoiding shunting or craniotomy. We consider that it is appropriate to use the rigid endoscope via the frontal approach. It is helpful to fill the ventricles with lactated Ringer's solution and leave an external drain after surgery.