Problems of diagnosis and treatment in the Dandy-Walker syndrome

10 children with Dandy-Walker syndrome are presented to discuss their clinical assessment and therapy. The presence on physical examination of a large posterior fossa was the most reliable clinical finding and was diagnostic in 9 out of the 10 patients. The different diagnostic confirmatory studies are discussed. The most satisfactory treatment in this series was the combination of a shunt from the lateral ventricle and the posterior fossa to the peritoneum.