BACKGROUND Choroid plexus papillomas are rare, accounting for less than 1% of all intracranial tumours in adults. However, they are relatively more common in childhood and constitute 1.5 to 4% of intracranial tumours. They are most often located in the lateral ventricle, followed by the fourth and third ventricles and, rarely, in the cerebellopontine angle. Bilateral lateral ventricle choroid plexus papilloma is very rare and only a few cases has been reported. METHODS A 3-year-old boy was admitted to our hospital with a history of irritability and vomiting. Neurological examination on admission was normal. A head computed tomographic scan and magnetic resonance imaging showed tumours in both lateral ventricles, hydrocephalus and a left temporal arachnoid cyst. The bilateral intraventricular mass enhanced densely and homogeneously. A presumptive diagnosis of choroid plexus papillomas was made. CONCLUSIONS The initial surgery was performed for removal of the lesion in the right lateral ventricle, and 20 days later removal of the left lateral ventricle tumour was carried out. Bilateral temporoparietal craniotomy and total removal of tumours was performed. Hydrocephalus was controlled by total tumour resection from both sides. The histology of these tumours was the same and revealed choroid plexus papilloma. Interpretation: Bilateral choroid plexus papilloma is extremely rare and distinct from diffuse villous hypertrophy and their surgical approaches are different from each other. Differential diagnosis should be made by MRI preoperatively. If bilateral choroid plexus papilloma is detected, total surgical resection should be performed. Total surgical removal of the neoplasm not only cures the tumour but also may lead to complete resolution of the hydrocephalus.