Larsen's syndrome is characterised by multiple joint dislocations, fla
t face and talipes equinovarus. There is an autosomal dominant form an
d also a more severe autosomal recessive form. Several types of polycy
stic kidney disease have been reported in children. In this report we
present an infant with a severe form of Larsen's syndrome (thought to
be lethal Larsen-like), infantile-type polycystic kidney disease, bili
ary dysgenesis and osteosclerosis.