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A female in her late 40s presented with progressively worsening headaches, nausea, vomiting and gait imbalance and was ultimately found to have a fourth ventricular mass and cervical cyst. Initially presumed to be a neoplastic process, histopathology revealed longstanding neurocysticercosis and reactive changes. The patient underwent suboccipital craniotomy, C1 laminectomy and external ventricular drain placement. Postoperative complications included persistent very low-pressure hydrocephalus (VLPH), venous sinus thrombosis and fluctuating mental status requiring prolonged active cerebrospinal fluid drainage and ventriculoperitoneal shunt placement. This case highlights the diagnostic and management challenges of chronic neurocysticercosis in a posterior fossa location, complicated by rare, VLPH.