Choroid plexus carcinoma is a rare intracranial neoplasm, affecting mainly very young children. The commonest site is within the lateral ventricles and the prognosis is very poor. We report a seven month old baby boy who presented with raised intracranial pressure and seizures. Brain CT scan showed large intraventricular mass with calcification and hydrocephalus. Total macroscopic resection of the tumour was performed and diagnosis of choroid plexus carcinoma was made. However, the patient died 11 days after the tumour excision. The histopathology of this rare childhood neoplasm is discussed.