Three cases are reported of hydrocephalus associated with spinal tumours. In two cases symptoms and signs of raised intracranial pressure and their spinal tumours developed in co-existence, in the third case hydrocephalus and multiple cerebral metastases developed ten years after removal of a thoraco-lumbal ependymoma. Pathophysiological aspects of concerted action of this rare entity and consequences of misdiagnosis are discussed, and the relevant literature is reviewed.