Asymptomatic cysts of the pineal region are common incidental findings in adults. In contrast, symptomatic pineal cysts are rare and their management is not well defined. We present the case of a 39-year-old woman suffering from intracranial hypertension, with visual disturbance and mild papilledema. The MR images showed a voluminous cyst of the pineal region responsible for an obstructive hydrocephalus. Endoscopic treatment with the aid of computerized neuronavigation consisting in third ventriculostomy and fenestration of the cyst was performed. Intracranial hypertension symptoms resolved in 24 hours. The one year follow-up cerebral MR images demonstrated the normalization of ventricular size with patency of the aqueduct of Sylvius. Reviewing the literature demonstrates that the endoscopic approach represents a minimally invasive and safe procedure in the treatment of symptomatic pineal cysts.