Choroid plexus papillomas account for only up to five per cent of the intracranial tumours occurring in childhood, the published cases representing only a minority of the scattered clinical experience. A review of 265 published cases, including seventeen personal examples, showed that the majority of the childhood tumours are in the lateral ventricles and behave clinically like cases of infancy-onset hydrocephalus, but are often somewhat bizarre and frequently show identifiable features due largely to the tumour being physiologically active. The fourth ventricular tumours behave clinically like most other posterior fossa tumours. Almost all of the tumours are benign; only a few are histologically or clinically malignant, and seeding only occurs in occasional cases. If the tumours are recognized for what they are and special precautions are taken to compensate for their physiological behaviour and abolish for the frequently accompanying basal cistern block, choroid plexus papillomas could be one of the most rewarding childhood tumours to treat surgically.