The medical literature concerning neuropathological changes caused by hydrocephalus is reviewed. In both humans and experimental animals the ependyma suffers focal destruction, cerebral blood vessels are distorted and capillaries collapse, there is damage to axons and myelin in the periventricular white matter, and occasionally neurons suffer injury. The damage appears to result from mechanical distortion of the brain combined with impaired cerebral blood flow. If ventriculomegaly develops very early, foci of cortical dysgenesis may be the result. The character and distribution of pathological changes are dependent on the age at which hydrocephalus develops, the rate and magnitude of ventricular enlargement, and the duration of hydrocephalus. Diversionary shunting of cerebrospinal fluid can only incompletely reverse the damage and the potential for reversal diminishes as the duration of hydrocephalus increases.