Five patients with the Dandy-Walker syndrome had dysgenesis of the cerebellar vermis, cystic dilatation of the fourth ventricle, and a high position of the tentorium cerebelli. When only these features are present, the patient may lead a normal life. Additional defects usually account for the prominent clinical and pathological features of this syndrome. In this series, one patient had aqueductal stenosis, four had agenesis of the corpus callosum, two had hydrocephalus, one had cerebral abiotrophy, and one (a 72-year-old man) had no additional defects and no symptoms from his Dandy-Walker syndrome. An analysis of development and teratological considerations indicates that the Dandy-Walker and Arnold-Chiari malformations are complex disorders that have different causes and mechanisms and begin at different times in the emryonic period. The causes are still unknown.

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