An uncommon but well-recognized occurrence in patients with Dandy-Walk
er malformation is sudden unexpected death. The mechanism of demise ha
s not been established. We report three patients with Dandy-Walker mal
formation that experienced sudden unexpected death without uncal or to
nsillar herniation, the mechanism usually proposed for demise in such
situations. Our findings suggest the possibility of vascular compromis
e as the cause of the sudden unexpected death in these patients. Early
and effective relief of the pressure in the posterior fossa may preve
nt the occurrence of this catastrophic complication.