An unexpected finding at autopsy of almost complete agenesis of the cerebel
lum in an apparently functional, mentally subnormal 38-year-old man who die
d as the result of an accidental electrocution is reported. The posterior f
ossa was normal in appearance despite nearly complete absence of the cerebe
llum. A number of syndromes of cerebellar atrophy or dysgenesis have been r
eported, but congenital agenesis is considered a very rare condition. It do
es not resemble most common cerebellar malformations or acquired conditions
, especially in an adult, who apparently had reasonable motor and coordinat
ive function. The relevant literature is reviewed.