Abstract Objectives Monoventricular hydrocephalus (MH) is a rare condition in which the site of obstruction is located around one of the interventricular foramen. In this paper, the authors offer their experiences in the neuroendoscopic management of this uncommon type of hydrocephalus. Patients and methods The authors retrospectively reviewed 12 neuroendoscopic procedures performed between July 2003 and June 2011 with MH. Clinical and radiological charts were reviewed and analysed. Results The operative course is a simple and successful procedure, and the postoperative complaints are mild. The postoperative radiological findings showed maintenance of ventricular enlargement in four cases and a significant decrease in enlargement in eight patients. However, upon shifting of the septum pellucidum, the periventricular transudation disappeared in all patients. Conclusions Fenestration of the septum pellucidum by neuroendoscope is the best treatment for patients with monoventricular hydrocephalus. The approach for fenestration of the septum pellucidum is based on the experience of the neurosurgeon and the preoperative planning.