Neurocysticercosis is a rare disease, and no association with intraventricular cysts has been reported in Japan. We report a case of neurocysticercosis that presented the unique symptom of a fourth ventricular cyst. A 55-year-old man with a 7 year history of neurocysticercosis and hydrocephalus developed a positional headache and vomiting 3 months after an L-P shunt, and was admitted to our hospital. MRI showed multiple intraparenchymal cysts, ventricular enlargement and an intraventricular cyst in the fourth ventricle. The cyst and the cysticercus in the fourth ventricle were totally removed via the suboccipital approach. Histological examination showed characteristics of cysticercus. Two weeks after this surgery, V-P shunt with neuroendoscopical observation of the ventricle was performed. A small cyst was detected in the right lateral ventricle. The symptoms disappeared after surgery. Following surgery, a therapeutic course of albendazole was administered at a daily dose of 600 mg for 30 days. MRI after administration of the anticysticercal drug revealed reduction in the size of the cysts.