Choroid plexus carcinoma is a rare aggressive malignant epithelial neoplasm (WHO grade III) arising from choroid plexus epithelium. It commonly occurs in children arising in lateral ventricles. This tumour has a tendency for recurrence and metastatic dissemination along cerebrospinal fluid (CSF) pathway. We report a case of 11 month old boy presenting with hydrocephalus, irritability and vomiting. Contrast enhanced computed tomography (CECT) brain and magnetic resonance imaging (MRI) brain with contrast revealed a mass lesion within right lateral ventricle and hydrocephalus. Surgical resection was done and the excised tissue was sent for cytology and histopathological examination and a diagnosis of choroid plexus carcinoma was made.