Objective: Central neurocytoma is a rare intraventricular brain tumor that affects young adults and presents with increased intracranial pressure secondary to obstructive hydrocephalus. They are usually located in the supratentorial periventricular region. Because of some clinical and radiological findings CNS neurocytomas were confused with other intraventricular lesions. Method: A 37-year-old male was admitted with chronic headache with 6 months duration on July 21, 2011, when there were no neurological deficits or physical abnormalities. Computed tomographic (CT) scans showed 51×40 mm a mixed density mass with amorphous calcification in the right lateral ventricle, which was irregularly enhanced by contrast medium. Light Microscopy showed sheets of monotonously neoplastic cells with uniform round-to-oval nuclei and inconspicuous nucleoli. The cytoplasm was clear or eosinophilic with indistinct border. Capillary networks were well developed and divided the tumor cells into groups. No nuclear pleomorphism or mitosis was seen. We present a case of intraventricular neurocytomas confirmed by immunohistochemical studies. Conclusion: This rare tumor causing diagnostic confusion, discussed by the literature.